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Journal articles on the topic 'Progressive hearing loss'

Author: Grafiati

Published: 25 May 2024

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1

Hayes, Deborah, and Susan Dreith. "Catastrophic Progressive Hearing Loss in Childhood." Journal of the American Academy of Audiology 11, no. 06 (2000): 300–308. http://dx.doi.org/10.1055/s-0042-1748058.

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AbstractWe present a report on a 5-year-old child with a complex medical and audiologic history who exhibited catastrophic progression in hearing loss. Hearing loss was initially attributed to bacterial meningitis at age 3 months; progression was apparently related to perilymph fistula at age 8 years. Etiologies associated with progressive hearing loss in children as well as signs of progression and monitoring protocols for children at risk for progressive hearing loss are discussed. Abbreviations: ABR = auditory brainstem response, CMV = cytomegalovirus, JCIH = Joint Committee on Infant Hearing, LVA = large vestibular aqueduct, PPHN = persistent pulmonary hypertension of the newborn, TCH = The Children's Hospital

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2

Fitzpatrick, Elizabeth M., Flora Nassrallah, Bénédicte Vos, JoAnne Whittingham, and Jessica Fitzpatrick. "Progressive Hearing Loss in Children With Mild Bilateral Hearing Loss." Language, Speech, and Hearing Services in Schools 51, no. 1 (2020): 5–16. http://dx.doi.org/10.1044/2019_lshss-ochl-19-0013.

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Purpose This study investigated progressive hearing loss in a cohort of children who were identified with permanent mild bilateral hearing loss. Method This population-based study included 207 children with permanent mild bilateral hearing loss, diagnosed and followed from 2003 to 2016 in 1 region of Canada. Clinical characteristics and initial audiologic results were collected prospectively at diagnosis, and audiologic information was updated. Changes in hearing levels between the 1st and most recent assessment were analyzed to determine progressive hearing loss. Clinical characteristics were compared between children with progressive and stable hearing loss. The association between risk indicators and progressive hearing loss was explored through logistic regression. Results A total of 47.4% (94 of 207) had progressive hearing loss in at least 1 ear, and 50% (47 of 94) of those experienced more than 20-dB average drop in thresholds. For these 94 children, a total of 147 ears were affected, and 116 (78.9%) ears experienced sufficient change in thresholds to be coded as a worse category of hearing loss. In the subset of 85 children with more than 5 years of audiologic follow-up, 56.4% (53/85) showed a decrease in hearing. Of the total sample of 207 children, 137 (66.2%) continued to have mild hearing loss in the better ear. There was no association between the risk factors examined (family history of hearing loss, admission to the neonatal intensive care unit, or presence of a syndrome) and progressive hearing loss. Conclusion This study found that almost half of children with mild bilateral hearing loss showed a decrease in hearing in at least 1 ear. One third of the children first diagnosed with mild hearing loss in the better ear now have moderate or worse hearing loss in both ears. These findings point to the importance of careful long-term monitoring of children who present with mild hearing loss.

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3

Roberts, Deanne M., Matthew L. Bush, and Raleigh O. Jones. "Adult Progressive Sensorineural Hearing Loss." Otology & Neurotology 35, no. 2 (2014): 241–45. http://dx.doi.org/10.1097/mao.0b013e3182a437b3.

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4

Whelan, Alison, and Anne Hing. "Genetics of Progressive Hearing Loss." Seminars in Hearing 16, no. 03 (1995): 246–56. http://dx.doi.org/10.1055/s-0028-1083722.

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5

Vrabec, Jeffrey T. "Hydrocodone Use and Sensorineural Hearing Loss." Pain Physician 3;10, no. 5;3 (2007): 467–72. http://dx.doi.org/10.36076/ppj.2007/10/467.

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Background: The hydrocodone/acetaminophen combination is one of the most commonly used analgesic preparations. Isolated incidences of suspected association between hydrocodone abuse and rapidly progressive hearing loss have been reported. In this study, we describe the clinical characteristics of 5 patients presenting with progressive hearing loss and a history of hydrocodone use. Methods: Patients presenting with rapidly progressive bilateral hearing loss who had a documented history of hydrocodone use were selected for the study. The presentation, audiologic findings, associated comorbidities, and treatment outcomes were reviewed Results: All patients displayed rapidly progressive sensorineural hearing loss without vestibular symptoms. Hearing loss was asymmetric in 3 patients at initial presentation, but progressed to profound loss, usually within months. Steroid treatment has no effect on the progression of the hearing loss. The admitted quantity of hydrocodone consumed ranged from 10 to 300 mg per day. Hepatitis C was the most common comorbidity, present in 60% of the patients. All patients underwent cochlear implantation with satisfactory results. Conclusions: The chronic use of hydrocodone can be associated with progressive sensorineural hearing loss. Successful auditory rehabilitation can be achieved with cochlear implantation. Genetic polymorphisms of drug metabolizing enzymes as well as associated comorbidities such as hepatitis C infection may be significant in the development of hydrocodone ototoxicity, though additional investigations are necessary. Key words: hydrocodone, sensorineural hearing loss, cochlear implant

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6

Levi, Haya, Lilly Tell, and Moshe Feinmesser. "Progressive Hearing Loss in Hard-of-Hearing Children." International Journal of Audiology 32, no. 2 (1993): 132–36. http://dx.doi.org/10.3109/00206099309071862.

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7

Hashimoto, Sho, and Harold F. Schuknecht. "Progressive Hearing Loss from Strial Dysplasia." Annals of Otology, Rhinology & Laryngology 96, no. 2 (1987): 229–31. http://dx.doi.org/10.1177/000348948709600219.

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An otherwise healthy male patient had a bilateral, slowly progressive hearing loss first noticed in early childhood and possibly present at birth. Audiometric studies at the age of 32 showed a moderately severe, bilateral, mixed type hearing loss. He died of unrelated causes at the age of 34. Studies of the temporal bones showed bilateral hypoplasia and atrophy of the striae vascularis as the cause of hearing loss. The history and findings are consistent with a genetically determined hypoplasia and atrophy of this structure.

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8

Karjalainen, Seppo, Leena Pakarinen, Helena Kääriäinen, Markku Teräsvirta, and Eero Vartiainen. "Progressive Hearing Loss in Usher's Syndrome." Annals of Otology, Rhinology & Laryngology 98, no. 11 (1989): 863–66. http://dx.doi.org/10.1177/000348948909801106.

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In 18 patients with Usher's syndrome, progressive hearing loss was verified audiologically in eight cases. Despite poor auditory threshold values and low speech discrimination scores, there was only one patient who could not communicate with speech. The possibility of hearing impairment being mainly progressive in Usher's syndrome is discussed.

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9

Lee, Jennifer W., and Manohar L. Bance. "Hearing loss." Practical Neurology 19, no. 1 (2018): 28–35. http://dx.doi.org/10.1136/practneurol-2018-001926.

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Hearing loss affects one in six people in the UK and is a significant disease burden. In addition to communication problems, there is also an association with depression and dementia. Clinical assessment with targeted history and examination can identify the characteristics and cause of hearing loss, and complementary audiological testing can confirm its type and severity. Retrocochlear screening is recommended for sudden, rapidly progressive or asymmetric sensorineural hearing loss. Medical or surgical therapies may be indicated in cases of conductive hearing loss, while hearing assistive devices and hearing aids are the mainstay of rehabilitation for sensorineural hearing loss.

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10

de Wolf, M. J. F., J. Honings, F. B. M. Joosten, L. Hoefsloot, E. A. M. Mylanus, and C. W. R. J. Cremers. "Two siblings with progressive, fluctuating hearing loss after head trauma, treated with cochlear implantation." Journal of Laryngology & Otology 124, no. 1 (2009): 86–89. http://dx.doi.org/10.1017/s0022215109990296.

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AbstractObjective:Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.Design:Case report.Subjects:Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.Results:These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good.Conclusion:Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.

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Reisser, Christoph F. V., William J. Kimberling, and Christian R. Otterstedde. "Hearing Loss in Usher Syndrome Type II is Nonprogressive." Annals of Otology, Rhinology & Laryngology 111, no. 12 (2002): 1108–11. http://dx.doi.org/10.1177/000348940211101208.

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Usher syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. In the literature, a possible progression of the moderate to severe hearing loss in Usher syndrome type II (Usher II) is controversial. We studied the development of the hearing loss of 125 patients with a clinical diagnosis of Usher syndrome type II intraindividually and interindividually by repeatedly performing complete audiological and neuro-otologic examinations. Our data show a very characteristic slope of the hearing curve in all Usher II patients and no clinically relevant progression of the hearing loss over up to 17 years. The subjective impression of a deterioration of the communicative abilities of Usher II patients must therefore be attributed to the progressive visual loss. The patients should be reassured that changes in their hearing abilities are unlikely and should be provided with optimally fitted modern hearing aids.

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Newton, V. E., and V. J. Rowson. "Progressive sensorineural hearing loss in childhood." British Journal of Audiology 22, no. 4 (1988): 287–95. http://dx.doi.org/10.3109/03005368809076466.

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Gray, R. F. "Cochlear implantation for progressive hearing loss." Archives of Disease in Childhood 88, no. 8 (2003): 708–11. http://dx.doi.org/10.1136/adc.88.8.708.

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Barreira-Nielsen, Carmen, Elizabeth Fitzpatrick, Serena Hashem, JoAnne Whittingham, Nicholas Barrowman, and Mary Aglipay. "Progressive Hearing Loss in Early Childhood." Ear and Hearing 37, no. 5 (2016): e311-e321. http://dx.doi.org/10.1097/aud.0000000000000325.

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15

Neely, J. "Overview of Progressive Sensorineural Hearing Loss." Seminars in Hearing 16, no. 03 (1995): 209–13. http://dx.doi.org/10.1055/s-0028-1083717.

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16

Berrettini, Stefano, Francesca Ravecca, Stefano Sellari-Franceschini, Fabio Matteucci, Gabriele Siciliano, and Francesco Ursino. "Progressive sensorineural hearing loss in childhood." Pediatric Neurology 20, no. 2 (1999): 130–36. http://dx.doi.org/10.1016/s0887-8994(98)00123-4.

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17

Meyerhoff, William L., Steven Cass, Mitchell K. Schwaber, Nancy Sculerati, and William H. Slattery. "Progressive Sensorineural Hearing Loss in Children." Otolaryngology–Head and Neck Surgery 110, no. 6 (1994): 569–79. http://dx.doi.org/10.1177/019459989411000617.

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18

Nishimura, Tadashi, and Hiroshi Hosoi. "Progressive hearing loss in intracochlear schwannoma." European Archives of Oto-Rhino-Laryngology 265, no. 4 (2007): 489–92. http://dx.doi.org/10.1007/s00405-007-0483-x.

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19

Marcos Alonso, Susana, Cristina Nicole Almeida Ayerve, Sofía Pacheco López, et al. "Hearing loss in vestibular schwannoma." Revista ORL 13, no. 1 (2022): e27266. http://dx.doi.org/10.14201/orl.27266.

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Purpose: The most common reason for consultation in patients suffering from Vestibular Schwannoma (VS) is progressive hearing loss. The main objective of this study is analyzing the hearing loss in patients with VS and determining the extent to which the tumor grade and the hearing loss are related. Methods: An observational retrospective study was conducted with a sample of 291 patients diagnosed with VS between 1995 and 2017 in the Department of Otorhinolaryngology and Head and Neck Surgery of the Tertiary Care Primary Center of Salamanca. We analyzed preoperative and postoperative data from patients with VS as well as radiological and surgical findings. Results: The most common reason for consultation at diagnosis was progressive unilateral hearing loss (31.6%). The relationship between the size of the VS and hearing loss in the patients who reported progressive unilateral hearing loss as a reason for consultation was not statistically significant (p=0.099). Conclusion: The most common reason for consultation in VS is progressive unilateral hearing loss. The hearing loss caused by VS does not have a statistically significant association with any particular tumor grade, although severe and profound hearing loss is more commonly associated with grade III-IV neurinomas, whereas mild hearing loss or normal hearing are more likely in grade I-II tumors.

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20

Lahiri, Asish Kumar, Anandita Gupta, Rakesh Vohra, Shalabh Sharma, and Satinder Singh. "Cochlear Otosclerosis: An Undiagnosed Cause of Progressive Sensorineural Hearing Loss." Bengal Journal of Otolaryngology and Head Neck Surgery 28, no. 2 (2020): 127–37. http://dx.doi.org/10.47210/bjohns.2020.v28i2.329.

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Introduction Otosclerosis presents as conductive or mixed hearing loss depending upon the stage of the disease. Isolated sensorineural hearing loss though known to occur has been rarely reported. Pure cochlear otosclerosis presenting as sensorineural hearing loss should be considered as a differential diagnosis in young patients presenting with progressive hearing loss. The aim of this article is to note the prevalence of cochlear otosclerosis in young patients presenting with progressive sensorineural hearing loss.  Materials and Methods Retrospective chart analysis of 19 patients who presented with progressive SNHL with unknown etiology was done. Results Otosclerosis involving the pericochlear region was diagnosed in 27 ears which was identified by high resolution CT scan of temporal bone. In four ears, in addition, there was demineralization just anterior to oval window without clinical evidence of stapes fixation. Conclusion In young patients with progressive SNHL, high resolution CT scan of temporal bone should be done to identify progressive cochlear otosclerosis. Medical management should be initiated in these patients to halt the progression of disease.

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21

Jenkinson, P. W., M. I. Syed, and L. Mcclymont. "Progressive, reversible sensorineural hearing loss caused by azathioprine." Journal of Laryngology & Otology 128, no. 9 (2014): 838–40. http://dx.doi.org/10.1017/s0022215114001807.

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AbstractObjective:This paper describes the first reported case of progressive sensorineural hearing loss caused by azathioprine, which was reversed on stoppage of the drug.Case report:A female patient with previously normal hearing presented with progressive sensorineural hearing loss after being started on azathioprine. Otological and neurological examination findings were unremarkable. After stopping the drug, the patient reported an improvement in hearing, which was confirmed on pure tone audiometry.Conclusion:This previously unreported side effect of azathioprine is highlighted in order to increase clinical awareness. Early recognition of this adverse effect is important to minimise the possibility of permanent sensorineural hearing loss.

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22

Potts, Lisa, and Michael Valente. "Programmable Hearing Aids: Applications for Progressive and Fluctuating Hearing Loss." Seminars in Hearing 16, no. 03 (1995): 266–79. http://dx.doi.org/10.1055/s-0028-1083724.

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23

Thalmann, Isolde, and J. Neely. "Progressive Hearing Loss and the Perilymphatic Fistula." Seminars in Hearing 16, no. 03 (1995): 214–18. http://dx.doi.org/10.1055/s-0028-1083718.

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24

Savastano, Marina, Marilena Savini, and Claudio Andreoli. "Idiopathic progressive sensorineural hearing loss in children." International Journal of Pediatric Otorhinolaryngology 26, no. 3 (1993): 225–33. http://dx.doi.org/10.1016/0165-5876(93)90092-h.

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25

Silkes, Ellen D., and Janie Chabot. "Progressive hearing loss following Haemophilus influenzae meningitis." International Journal of Pediatric Otorhinolaryngology 9, no. 3 (1985): 249–56. http://dx.doi.org/10.1016/s0165-5876(85)80041-0.

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26

Terayama, Yoshihiko, Yuhko Ishibe, and Jun-Ichi Matsushima. "Rapidly Progressive Sensorineural Hearing Loss (Rapid Deafness)." Acta Oto-Laryngologica 105, sup456 (1988): 43–48. http://dx.doi.org/10.3109/00016488809125076.

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Steel, K. P. "HUMAN GENETICS: Progress in Progressive Hearing Loss." Science 279, no. 5358 (1998): 1870–71. http://dx.doi.org/10.1126/science.279.5358.1870.

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28

Battelino, S., B. Repič Lampret, M. Žargi, and K. Trebušak Podkrajšek. "Novel connexin 30 and connexin 26 mutational spectrum in patients with progressive sensorineural hearing loss." Journal of Laryngology & Otology 126, no. 8 (2012): 763–69. http://dx.doi.org/10.1017/s0022215112001119.

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AbstractObjective:Mutations in the gap junction protein beta-2 gene (‘GJB2’) are known to be responsible for mild to profound congenital and late-onset hearing loss. This study aimed to investigate the molecular basis of progressive hearing loss compared with non-progressive hearing loss.Methods:Following clinical otorhinolaryngological evaluation, a genetic analysis was performed in a cohort of 72 patients with progressive sensorineural hearing loss.Results:Pathological genotypes were established in 16 patients (22.2 per cent). Six different gap junction protein beta-2 gene mutations were detected in 15 patients, with the c.35delG mutation responsible for 56 per cent of the mutated alleles. A novel gap junction protein beta-6 gene (‘GJB6’) mutation (p.Met203Val) was observed in one patient with mild progressive hearing loss.Conclusion:Analyses of gap junction protein beta-2 and -6 genes revealed that similar pathological genotypes, occurring with similar frequencies, were responsible for progressive hearing loss, compared with reported genotypes for non-progressive hearing loss patients. Thus, genotype cannot be used to differentiate non-progressive from progressive hearing loss cases; in this study, patients both with and without an established pathological genotype had a similar clinical course.

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Hiraki, N., T. Udaka, H. Yamamoto, Y. Kadokawa, J. Ohkubo, and H. Suzuki. "Mitochondrial neurogastrointestinal encephalomyopathy associated with progressive hearing loss." Journal of Laryngology & Otology 124, no. 9 (2010): 1007–9. http://dx.doi.org/10.1017/s0022215110001477.

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AbstractObjective:We report a rare case of mitochondrial neurogastrointestinal encephalomyopathy with hearing loss.Case report:A 46-year-old woman presented with a three-year history of progressive, bilateral hearing loss and tinnitus. She had been suffering from unexplained abdominal pain and diarrhoea for 20 years. When first seen, her otoscopic findings were normal, and pure tone audiometry showed mild and moderate hearing loss in her right and left ears, respectively. She also had: bilateral ophthalmoparesis, neck and limb muscle weakness, and hypoactive deep tendon reflexes on neurological examination; diffuse leukoencephalopathy on magnetic resonance imaging of the brain; and markedly reduced leukocyte thymidine phosphorylase activity. On the basis of these findings, the patient was diagnosed with mitochondrial neurogastrointestinal encephalomyopathy.Conclusion:Mitochondrial neurogastrointestinal encephalomyopathy is an autosomal recessive disease caused by mutation of the thymidine phosphorylase gene, and is characterised by ophthalmoparesis, peripheral neuropathy, leukoencephalopathy, gastrointestinal symptoms and abnormal mitochondria in muscle cells. Current advances in genetic research may reveal a higher prevalence of mitochondrial disorders than had previously been thought. Otolaryngologists should be aware of mitochondrial neurogastrointestinal encephalomyopathy and other rare genetic disorders when managing patients with progressive hearing loss.

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NIELD, TOM, ANGELA D. RAMOS, and DAVID WARBURTON. "Late-Onset Hearing Loss." Pediatrics 83, no. 5 (1989): 807–8. http://dx.doi.org/10.1542/peds.83.5.807a.

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The findings of Hendricks-Munoz and Walton concerning the incidence of sensorineural hearing loss in infants with persistent pulmonary hypertension (persistent fetal circulation) substantiate our findings that, in some cases, the loss appears to be of delayed onset and progressive. We would, however, like to point out that delayed-onset sensorineural loss is not limited to infants with persistent pulmonary hypertension, because four of the 11 infants we reported did not have persistent pulmonary hypertension. Therefore, serial audiologic evaluations should also be done for any infant with significant respiratory failure requiring prolonged mechanical ventilation with resultant chronic lung disease.

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Branch, Matthew P., and Barry E. Hirsch. "Management of Unilateral Acoustic Neuroma in a 72-Year-Old Patient with Contralateral Congenital Deafness." Otolaryngology–Head and Neck Surgery 127, no. 5 (2002): 483–86. http://dx.doi.org/10.1067/mhn.2002.129809.

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Patients with asymmetric or progressive hearing impairment warrant evaluation for the cause of their loss. We present a 72–year–old woman with near life–long hearing loss in one ear and a history of contralateral slowly progressive hearing loss. The audiometric and radiologic findings are demonstrated along with discussion of the management of acoustic neuroma in an only hearing ear.

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Mori, Kentaro, Ikuyo Miyanohara, Hideaki Moteki, Shin-ya Nishio, Yuichi Kurono, and Shin-ichi Usami. "Novel Mutations in GRXCR1 at DFNB25 Lead to Progressive Hearing Loss and Dizziness." Annals of Otology, Rhinology & Laryngology 124, no. 1_suppl (2015): 129S—134S. http://dx.doi.org/10.1177/0003489415575061.

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Objective: We identified 2 patients in 1 family who had novel mutations in GRXCR1, which caused progressive hearing loss. Methods: One thousand one hundred twenty Japanese hearing loss patients with sensorineural hearing loss from unrelated families were enrolled in this study. Targeted genomic enrichment with massively parallel sequencing of all known nonsyndromic hearing loss genes was used to identify the genetic causes of hearing loss. Results: In this study, 2 affected individuals with compound heterozygous mutations—c.439C>T (p.R147C) and c.784C>T (p.R262X)—in GRXCR1 were identified. The proband had moderate to severe hearing loss and suffered from dizziness with bilateral canal paralysis. Conclusion: Our cases are the first identified in the Japanese population and are consistent with previously reported cases. The frequency of mutations in GRXCR1 seems to be extremely rare. This study underscores the importance of using comprehensive genetic testing for hearing loss. Furthermore, longitudinal audiologic assessment and precise vestibular testing are necessary for a better understanding of the mechanisms of hearing loss and vestibular dysfunction caused by GRXCR1 mutations.

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Gilroy, Patricia A., and Carl L. Reams. "Delayed Progressive Sensorineural Hearing Loss after Electrocution Injury." Otolaryngology–Head and Neck Surgery 129, no. 2 (2003): P262. http://dx.doi.org/10.1016/s0194-59980301191-4.

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Ichijo, Hiroaki, Shin-Ichi Saito, Shigetoshi Fujita, and Hideichi Shinkawa. "Vestibular Function in Bilateral Progressive Sensorineural Hearing Loss." Acta Oto-Laryngologica 115, sup519 (1995): 193–96. http://dx.doi.org/10.3109/00016489509121902.

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HICKSON, L. M. H., and D. ALCOCK. "Progressive hearing loss in children with congenital cytomegalovirus." Journal of Paediatrics and Child Health 27, no. 2 (1991): 105–7. http://dx.doi.org/10.1111/j.1440-1754.1991.tb00362.x.

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Kenna, Margaret A., and Nancy Sculerati. "Management of Progressive Sensorineural Hearing Loss in Children." Otolaryngology–Head and Neck Surgery 112, no. 5 (1995): P117. http://dx.doi.org/10.1016/s0194-5998(05)80294-3.

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Educational objectives: To cite the differential diagnosis of progressive sensorineural hearing loss in children and to provide a reasonable algorithm of both medical and surgical treatment modalities for this condition.

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Gilroy, P. "Delayed progressive sensorineural hearing loss after electrocution injury." Otolaryngology - Head and Neck Surgery 129, no. 2 (2003): P262. http://dx.doi.org/10.1016/s0194-5998(03)01191-4.

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Koitschev, Assen, Katharina Gramlich, Sandra Hansmann, et al. "Progressive familial hearing loss in Muckle-Wells syndrome." Acta Oto-Laryngologica 132, no. 7 (2012): 756–62. http://dx.doi.org/10.3109/00016489.2012.656321.

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Ravecca, Francesca, Stefano Berrettini, Francesca Forli, et al. "Cx26 Gene Mutations in Idiopathic Progressive Hearing Loss." Journal of Otolaryngology 34, no. 02 (2005): 126. http://dx.doi.org/10.2310/7070.2005.04017.

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40

Lyos, A. T., M. A. Marsh, H. A. Jenkins, and N. J. Coker. "Progressive Hearing Loss After Transverse Temporal Bone Fracture." Archives of Otolaryngology - Head and Neck Surgery 121, no. 7 (1995): 795–99. http://dx.doi.org/10.1001/archotol.1995.01890070081017.

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Fuse, Takeo, and Margareta B. Møller. "Delayed and Progressive Hearing Loss after Microvascular Decompression of Cranial Nerves." Annals of Otology, Rhinology & Laryngology 105, no. 2 (1996): 158–61. http://dx.doi.org/10.1177/000348949610500212.

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An unusual case of unilateral delayed and progressive hearing loss following a microvascular decompression operation on cranial nerves V, VII, and VIII on the left side is reported. Preoperative and postoperative audiologic evaluation revealed a mild high-frequency hearing loss for both ears, normal thresholds for the acoustic middle ear reflex response, and normal brain stem auditory evoked potentials. Three years after this microvascular decompression procedure, the patient noticed slowly decreasing hearing in her left ear, and subsequent serial audiograms revealed a progressive sensorineural hearing loss and a decrease in her speech discrimination score. Brain stem auditory evoked potentials showed progressive changes. Because of the patient's increasing symptoms of vertigo and tinnitus in the left ear, reexploration of the eighth cranial nerve was performed 5½ years after the initial procedure. This second operation revealed reactive tissue around the eighth cranial nerve that was atrophic and yellow. We interpret the delayed and progressive hearing loss to be a result of reactive scar tissue and progressive atrophy of the auditory nerve.

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van Aarem, Annelies, Alfred J. L. G. Pinckers, William J. Kimberling, Patrick L. M. Huygen, Elisabeth M. Bleeker-Wagemakers, and W. R. J. Cremers. "Stable and Progressive Hearing Loss in Type 2A Usher's Syndrome." Annals of Otology, Rhinology & Laryngology 105, no. 12 (1996): 962–67. http://dx.doi.org/10.1177/000348949610501206.

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Audiograms were traced or additionally performed on 23 Usher's syndrome patients in 10 Dutch multi-affected families, all linked to chromosome 1q (USH2A locus). Serial audiograms, available in 13 patients, were used for a regression analysis of binaural pure tone average on age (follow-up, 9 to 32 years) to test for “significant progression,” ie, a significant regression coefficient, here called the “annual threshold increase” (ATI, expressed in decibels per year). A significant ATI (>1 dB/y) was observed in 3 patients. Analysis of variance of ATI demonstrated significant heterogeneity; hearing loss was either stable or progressive. This implies a significant clinical heterogeneity. A similar analysis performed on our progressive USH2A cases and “type III” cases previously reported by others (ATI of 1 to 5 dB/y), some of which were recently linked to chromosome 3q (USH3 locus), failed to show any significant heterogeneity in the progression of hearing loss.

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Nakashima, Takahiro, Akira Ganaha, Shougo Tsumagari, et al. "Is the Conductive Hearing Loss in NOG-Related Symphalangism Spectrum Disorder Congenital?" ORL 83, no. 3 (2021): 196–202. http://dx.doi.org/10.1159/000512668.

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We describe a dominant Japanese patient with progressive conductive hearing loss who was diagnosed with <i>NOG</i>-related symphalangism spectrum disorder (<i>NOG</i>-SSD), a spectrum of congenital stapes fixation syndromes caused by <i>NOG</i> mutations. Based on the clinical features, including proximal symphalangism, conductive hearing loss, hyperopia, and short, broad middle, and distal phalanges of the thumbs, his family was diagnosed with stapes ankylosis with broad thumbs and toes syndrome (SABTT). Genetic analysis revealed a heterozygous substitution in the <i>NOG</i> gene, c.645C&#x3e;A, p.C215* in affected family individuals. He had normal hearing on auditory brainstem response (ABR) testing at ages 9 months and 1 and 2 years. He was followed up to evaluate the hearing level because of his family history of hearing loss caused by SABTT. Follow-up pure tone average testing revealed the development of progressive conductive hearing loss. Stapes surgery was performed, and his post-operative hearing threshold improved to normal in both ears. According to hearing test results, the stapes ankylosis in our SABTT patient seemed to be incomplete at birth and progressive in early childhood. The ABR results in our patient indicated the possibility that newborn hearing screening may not detect conductive hearing loss in patients with <i>NOG</i>-SSD. Hence, children with a family history and/or known congenital joint abnormality should undergo periodic hearing tests due to possible progressive hearing loss. Because of high success rates of stapes surgeries in cases of SABTT, early surgical interventions would help minimise the negative effect of hearing loss during school age. Identification of the nature of conductive hearing loss due to progressive stapes ankylosis allows for better genetic counselling and proper intervention in <i>NOG</i>-SSD patients.

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Sakuma, Naoko, Hideaki Moteki, Hela Azaiez, et al. "Novel PTPRQ Mutations Identified in Three Congenital Hearing Loss Patients With Various Types of Hearing Loss." Annals of Otology, Rhinology & Laryngology 124, no. 1_suppl (2015): 184S—192S. http://dx.doi.org/10.1177/0003489415575041.

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Objectives: We present 3 patients with congenital sensorineural hearing loss (SNHL) caused by novel PTPRQ mutations, including clinical manifestations and phenotypic features. Methods: Two hundred twenty (220) Japanese subjects with SNHL from unrelated and nonconsanguineous families were enrolled in the study. Targeted genomic enrichment with massively parallel DNA sequencing of all known nonsyndromic hearing loss genes was performed to identify the genetic cause of hearing loss. Results: Four novel causative PTPRQ mutations were identified in 3 cases. Case 1 had progressive profound SNHL with a homozygous nonsense mutation. Case 2 had nonprogressive profound SNHL with a compound heterozygous mutation (nonsense and missense mutation). Case 3 had nonprogressive moderate SNHL with a compound heterozygous mutation (missense and splice site mutation). Caloric test and vestibular evoked myogenic potential (VEMP) test showed vestibular dysfunction in Case 1. Conclusion: Hearing loss levels and progression among the present cases were varied, and there seem to be no obvious correlations between genotypes and the phenotypic features of their hearing loss. The PTPRQ mutations appeared to be responsible for vestibular dysfunction.

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Kang, Minjin, Jung Ah Kim, Mee Hyun Song, et al. "Novel Variant in CEP250 Causes Protein Mislocalization and Leads to Nonsyndromic Autosomal Recessive Type of Progressive Hearing Loss." Cells 12, no. 18 (2023): 2328. http://dx.doi.org/10.3390/cells12182328.

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Genetic hearing loss is the most common hereditary sensorial disorder. Though more than 120 genes associated with deafness have been identified, unveiled causative genes and variants of diverse types of hearing loss remain. Herein, we identified a novel nonsense homozygous variant in CEP250 (c.3511C>T; p.Gln1171Ter) among the family members with progressive moderate sensorineural hearing loss in nonsyndromic autosomal recessive type but without retinal degeneration. CEP250 encodes C-Nap1 protein belonging to the CEP protein family, comprising 30 proteins that play roles in centrosome aggregation and cell cycle progression. The nonsense variant in CEP250 led to the early truncating protein of C-Nap1, which hindered centrosome localization; heterologous expression of CEP250 (c.3511C>T) in NIH3T3 cells within cilia expression condition revealed that the truncating C-Nap1 (p.Gln1171Ter) was not localized at the centrosome but was dispersed in the cytosol. In the murine adult cochlea, Cep250 was expressed in the inner and outer hair cells. Knockout mice of Cep250 showed significant hair cell degeneration and progressive hearing loss in auditory brainstem response. In conclusion, a nonsense variant in CEP250 results in a deficit of centrosome localization and hair cell degeneration in the cochlea, which is associated with the progression of hearing loss in humans and mice.

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Levy, Charles E., Albert T. Lash, Hal B. Janoff, and Frederick S. Kaplan. "Conductive Hearing Loss in Individuals with Fibrodysplasia Ossificans Progressiva." American Journal of Audiology 8, no. 1 (1999): 29–33. http://dx.doi.org/10.1044/1059-0889(1999/011).

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Fibrodysplasia ossificans progressiva (FOP) is a very rare genetic disorder that is characterized by progressive heterotopic ossification of soft tissues and congenital malformation of the great toes. Although previous case studies have reported hearing loss in individuals with FOP, there have been no large-scale studies regarding the nature or cause of the hearing loss. Here, we report the findings of a two-part study. In Part I, we report the findings of a postal survey regarding hearing loss that was sent to 102 individuals with FOP. In Part II, we report the findings of on-site hearing evaluations of eight individuals with FOP. The findings of both studies indicate that individuals with FOP are at risk for hearing loss and that the type of loss is predominantly conductive in nature, similar to that seen in individuals who have otosclerosis.

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Dahle, Arthur J., Karen B. Fowler, John D. Wright, Suresh B. Boppana, William J. Britt, and Robert F. Pass. "Longitudinal Investigation of Hearing Disorders in Children with Congenital Cytomegalovirus." Journal of the American Academy of Audiology 11, no. 05 (2000): 283–90. http://dx.doi.org/10.1055/s-0042-1748054.

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AbstractThis investigation consisted of a longitudinal study of the effects of congenital cytomegalovirus (CMV) infection on hearing sensitivity in 860 children with documented asymptomatic or symptomatic congenital CMV infection. Of the 651 children with asymptomatic CMV infection, 48 (7.4%) developed sensorineural hearing loss (SNHL), compared to 85 (40.7%) of the children with symptomatic CMV infection. Children in both groups experienced latent effects consisting of delayed onset of loss, threshold fluctuations, and/or progressive loss of hearing. It can be concluded that congenital CMV infection is a leading cause of SNHL in children. The late onset and progression of loss necessitates continued monitoring of hearing sensitivity in this population. Abbreviations: ABR = auditory brainstem response, CMV = cytomegalovirus, OAEs = otoacoustic emissions, SNHL = sensorineural hearing loss

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Hiramatsu, Ken, Shin-ya Nishio, Shin-ichiro Kitajiri, Tomohiro Kitano, Hideaki Moteki, and Shin-ichi Usami. "Prevalence and Clinical Characteristics of Hearing Loss Caused by MYH14 Variants." Genes 12, no. 10 (2021): 1623. http://dx.doi.org/10.3390/genes12101623.

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Variants in MYH14 are reported to cause autosomal dominant nonsyndromic hereditary hearing loss (ADNSHL), with 34 variants reported to cause hearing loss in various ethnic groups. However, the available information on prevalence, as well as with regard to clinical features, remains fragmentary. In this study, genetic screening for MYH14 variants was carried out using a large series of Japanese hearing-loss patients to reveal more detailed information. Massively parallel DNA sequencing of 68 target candidate genes was applied in 8074 unrelated Japanese hearing-loss patients (including 1336 with ADNSHL) to identify genomic variations responsible for hearing loss. We identified 11 families with 10 variants. The prevalence was found to be 0.14% (11/8074) among all hearing-loss patients and 0.82% (11/1336) among ADNSHL patients. Nine of the eleven variants identified were novel. The patients typically showed late-onset hearing loss arising later than 20 years of age (64.3%, 9/14) along with progressive (92.3%, 12/13), moderate (62.5%, 10/16), and flat-type hearing loss (68.8%, 11/16). We also confirmed progressive hearing loss in serial audiograms. The clinical information revealed by the present study will contribute to further diagnosis and management of MYH14-associated hearing loss.

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Tsukada, Keita, Aya Ichinose, Maiko Miyagawa, et al. "Detailed Hearing and Vestibular Profiles in the Patients with COCH Mutations." Annals of Otology, Rhinology & Laryngology 124, no. 1_suppl (2015): 100S—110S. http://dx.doi.org/10.1177/0003489415573074.

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Objectives: To evaluate the clinical features of Japanese DFNA9 families with mutations of the COCH gene. Methods: Mutation screening was performed using targeted next-generation sequencing (NGS) for 63 previously reported deafness genes. The progression of hearing loss and vestibular dysfunction were evaluated by pure-tone audiometry, caloric testing, cVEMP, and computed dynamic posturography. Results: We detected 1 reported mutation of p.G88E and 2 novel mutations of p.I372T and p.C542R. The patients with the novel mutations of p.I372T and p.C542R within the vWFA2 domain showed early onset progressive hearing loss, and the patients with the p.G88E mutation showed late onset hearing loss and acute hearing deterioration over a short period. Vestibular symptoms were reported in the patients with p.G88E and p.C542R. Vestibular testing was performed for the family with the p.G88E mutation. Severe vestibular dysfunction was observed in the proband, and the proband’s son showed unilateral semicircular canal dysfunction with mild hearing loss. Conclusions: Targeted exon resequencing of selected genes using NGS successfully identified mutations in the relatively rare deafness gene, COCH, in the Japanese population. The phenotype is compatible with that described in previous reports. Additional supporting evidence concerning progressive hearing loss and deterioration of vestibular function was obtained from our study.

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Alhamadani, Anmar Abdullah Jassim, Ghassan Mohammad Ahmed Alrawy, Ahmed Saadallah Ahmed Alhankawi, and Samet Almoula. "Sensorineural Hearing Loss in Diabetes Mellitus." Kufa Medical Journal 19, no. 1 (2023): 18–24. http://dx.doi.org/10.36330/kmj.v19i1.11076.

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Background: Sensorineural hearing loss are common neurological deficit, and diabetes mellitus is a common cause for these problems. Hearing impairment affects the quality of life of diabetic patients. Many researchers found hearing impairment in diabetic patients because diabetes mellitus can cause sensorineural hearing loss (SNHL) because of neuropathy and microvascular complications of diabetes mellitus. Aim of the study: The study aims to sensorineural hearing loss in diabetic patients compared with non-diabetic control. Patients and methods: A prospective case control study is performed during the period between Jan 2018 and May 2019. This case series study included 100 known cases of diabetes mellitus, their ages were between 20-60 years, treated at Al-wafaa center, which is specialized center for treatment and follow up of diabetic patients, and Al-Jumhory Teaching Hospital in Mosul. The control group included 100 individuals who did not have any diabetes mellitus or any of the exclusion criteria mentioned in the study. Results: It has been found that sensorineural hearing loss (SNHL) was more common in diabetic patients group than controls group, 21% versus 8%. The prevalence of (SNHL) was more in male 31.7% than female diabetic patients 13.5%. Hearing threshold in diabetic patients was higher than that in control group. The hearing loss in diabetic patients is slowly progressive and directly related to the duration of diabetes. Conclusions: Sensorineural hearing loss has been found to be more common in diabetic patients than in control group, and the hearing loss in diabetic patients is slowly progressive. The hearing threshold in diabetic patients had a direct correlation with the duration of disease.

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