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1

International Wullstein Symposium (1989 Würzburg, Germany). Sensorineural hearing loss and equilibrium disturbances. Stuttgart ; New York: Georg Thieme Verlag ; New York : Thieme Medical Publishers, 1990.

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2

Reger, Conference (1984 Iowa City Iowa). Sensorineural hearing loss: Mechanisms, diagnosis, treatment. Iowa City: University of Iowa, 1986.

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3

Reger Conference (1984 Iowa City, Iowa). Sensorineural hearing loss: Mechanisms, diagnosis, treatment. Iowa City: University of Iowa, 1986.

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4

White, Karl. Implementing universal newborn hearing screening programs: Early identification of hearing loss. [Rockville, MD]: Division of Services for Children with Special Health Needs, Maternal and Child Health Bureau, U.S. Dept. of Health & Human Services, Public Health Service, Health Resources & Services Administration, 1999.

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5

Hearing in aging. San Diego, Calif: Singular Pub. Group, 1995.

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6

Annemarie, Sommer, ed. Handbook of congenital and early onset hearing loss. New York: Igaku-Shoin, 1991.

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7

1935-, Honjō Iwao, and Takahashi H. 1952-, eds. Cochlear implant and related sciences update: 1st Asia Pacific Symposium on Cochlear Implant and Related Sciences, Kyoto, April 3-5, 1996. Basel: Karger, 1997.

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8

Razi, Mohammad Shakil. Cause of bilateral sensorineural hearing loss in school children in Karachi, Pakistan. Manchester: University of Manchester, 1994.

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9

Diagnosis and treatment of hearing impairment in children. 2nd ed. San Diego, Calif: Singular Pub. Group, 1998.

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10

Diagnosis and treatment of hearing impairment in children: A clinical manual. San Diego, Calif: College-Hill Press, 1985.

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11

I, Berlin Charles, and Wenthold Robert, eds. Neurotransmission and hearing loss: Basic science, diagnosis, and management : proceedings of the Second Annual Kresge-Mirmelstein Symposium in honor of Robert Wenthold, held in New Orleans, September 29, 1995. San Diego: Singular Pub. Group, 1997.

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12

Neurootological and Equilibriometric Society. Scientific Meeting. Conservative versus surgical treatment of sensorineural hearing loss, tinnitus, vertigo and nausea: Proceedings of the XVIIIth Scientific Meeting of the Neurootological and Equilibriometric Society (NES), Budapest, Hungary, 4-7 April 1991. Edited by Claussen Claus-Frenz, Kirtane Milind V, and Schneider Dieter. Hamburg: Edition M + P, 1992.

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13

Wolstenholme, G. E. W., and Julie Knight. Sensorineural Hearing Loss. Wiley & Sons, Incorporated, John, 2009.

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14

Jesteadt, Walt. Modeling Sensorineural Hearing Loss. Taylor & Francis Group, 2015.

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15

Walt, Jesteadt, ed. Modeling sensorineural hearing loss. Mahwah, N.J: Lawrence Erlbaum Associates, 1997.

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16

Lambert, Paul, and Jonathan Hatch. Sudden sensorineural hearing loss. Edited by John Phillips and Sally Erskine. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198834281.003.0026.

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17

Kennedy, Veronica. Paediatric sensorineural hearing loss. Edited by John Phillips and Sally Erskine. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198834281.003.0074.

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18

Jesteadt, Walt, ed. Modeling Sensorineural Hearing Loss. Routledge, 2019. http://dx.doi.org/10.4324/9781315789392.

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19

Helms, J. Sensorineural Hearing Loss and Equilibrium Disturbances. Thieme Publishing Group, 1990.

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20

Sensorineural Hearing Loss: Pathophysiology, Diagnosis and Treatment. Nova Science Publishers, Incorporated, 2019.

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21

Barton, Edmund. Sensorineural Hearing Loss: Prevalence, Risk Factors and Treatment. Nova Science Publishers, Incorporated, 2018.

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22

Ramsden, James. Hearing loss. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0051.

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Hearing loss must be divided into conductive hearing loss (CHL) and sensorineural hearing loss (SNHL). CHL is caused by sound not reaching the cochlear (abnormality of the ear canal, tympanic membrane, middle ear, or ossicles), whereas SNHL is a condition affecting the cochlear or auditory (eighth cranial) nerve. Hearing loss may be accompanied by other cardinal signs of ear disease, such as pain or discharge from the ear, vertigo, facial nerve palsy, and tinnitus, which guide the diagnosis. This chapter describes the approach to the patient with hearing loss.
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23

United States. Maternal and Child Health Bureau. Division of Services for Children with Special Health Needs, United States. Health Resources and Services Administration., and United States. Maternal and Child Health Bureau., eds. Implementing universal newborn hearing screening programs: Early identification of hearing loss. [Rockville, MD]: Division of Services for Children with Special Health Needs, Maternal and Child Health Bureau, U.S. Dept. of Health & Human Services, Public Health Service, Health Resources & Services Administration, 1999.

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24

Willems, Patrick J. Genetic Hearing Loss. Taylor & Francis Group, 2003.

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25

Willems, Patrick J. Genetic Hearing Loss. Taylor & Francis Group, 2003.

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26

Willems, Patrick J. Genetic Hearing Loss. Taylor & Francis Group, 2003.

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27

Willems, Patrick J. Genetic Hearing Loss. Taylor & Francis Group, 2003.

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28

Genetic Hearing Loss. Marcel Dekker, 2003.

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29

Molecular Mechanisms of Sensorineural Hearing Loss and Development of Inner Ear Therapeutics. MDPI, 2021. http://dx.doi.org/10.3390/books978-3-0365-1505-2.

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30

Hören und Gleichgewicht: Im Blick des gesellschaftlichen Wandels ; 7. Hennig-Symposium, Heidelberg. Wien: Springer, 2010.

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31

J, Judd Sandra, ed. Ear, nose, and throat disorders sourcebook: Basic consumer health information about disorders of the ears, hearing loss, vestibular disorders, nasal and sinus problems, throat and vocal cord disorders, and otolaryngologic cancers, including facts about ear infections and injuries, genetic and congenital deafness, sensorineural hearing disorders, tinnitus, vertigo, ménière disease, rhinitis, sinusitis, snoring, sore throats, hoarseness, and more; along with reports on current research initiatives, a glossary of related medical terms, and a directory of sources for further help and information. 2nd ed. Detroit, MI: Omnigraphics, 2006.

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32

Publications, ICON Health. The Official Patient's Sourcebook on Sudden Sensorineural Hearing Loss: A Revised and Updated Directory for the Internet Age. ICON Health Publications, 2006.

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33

Patterson, Marc C. Congenital Disorders of Glycosylation. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0066.

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Congenital disorders of glycosylation (CDG) comprise a family of multisystem diseases in which N- and O-linked glycosylation and glypiation of a variety of proteins and lipids is deficient. The hypoglycosylation of multiple glycoconjugates impairs normal development of the brain (and other organs), and is associated with both episodic and chronic organ dysfunction. Developmental disorders; seizures; strokelike episodes (and stroke); hearing and visual loss; peripheral neuropathy; coagulopathy; and immune, liver, endocrine, cardiac, and cutaneous manifestations may occur in varying combinations. Specific therapy is available for MPI-CDG and SLC35C2-CDG. Most forms of O-linked CDG affect muscle; these include congenital muscular dystrophies and limb girdle dystrophies.
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34

Elliman, David. Identification of hearing impairment. Edited by Alan Emond. Oxford University Press, 2019. http://dx.doi.org/10.1093/med/9780198788850.003.0020.

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Universal newborn hearing screening has meant that babies with significant congenital hearing impairment can be identified soon after birth and management instituted to ameliorate resultant problems, that is, minimize disability and handicap and optimize life chances. Evidence for the value of school entry screening is lacking and there is some evidence it is neither effective nor cost-effective. Further research is needed on this. Otitis media with effusion can cause significant long-lasting effects and may need surgical intervention or the provision of hearing aids. Parents and professionals should be aware of the symptoms that are indicative of possible hearing loss. Parents’ concerns should always be taken seriously.
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35

Dammeyer, Jesper. Mental Health and Psychosocial Well-Being in Deaf and Hard-of-Hearing Students. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780190880545.003.0021.

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This chapter considers two main explanations for the high prevalence of mental disorders among children with congenital deafness. The first is a medical explanation and focuses on the possibility of shared biologic causes for the hearing loss and the associated mental disorders. The second is multifactorial and focuses on interrelated psychological and social factors, in particular the psychosocial impact of a child’s delay in learning language. According to this account, hearing loss risks language delay, which in turn risks delays in social and cognitive development, which in turn increases the risk of mental disorders. The chapter goes on to discuss other risk and protective factors, including deaf identity, type of education, and cochlear implantation, as well as prevention and treatment services and programs.
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36

Mason, Peggy. Audition. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780190237493.003.0016.

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Hearing loss is devastating because it prevents communication through verbal language and thereby produces social isolation. The experience of hearing loss or deafness is the most common sensory deficit. The experience of affected individuals is highly variable because it depends on age of onset and treatment efficacy, among many factors. The roles of the external and middle ears in conduction and of the internal ear in sensorineural processing are used as a framework for understanding common forms of hearing loss. The contributions of inner and outer hair cells to cochlear function are detailed. How cochlear amplification results from the actions of prestin in outer hair cells is explained. The roles of age, noise, genetic background, and environmental factors in presbyacusis are considered. Approaches to hearing loss, including cochlear implants and sign language, are discussed. Finally, the brain regions involved in speech production and comprehension are detailed.
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37

Heidet, Laurence, Bertrand Knebelmann, and Marie Claire Gubler. Alport syndrome. Edited by Neil Turner. Oxford University Press, 2015. http://dx.doi.org/10.1093/med/9780199592548.003.0321.

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Alport syndrome is an inherited renal disorder characterized by early haematuria, progressing to proteinuria, sensorineural hearing loss, and progressive renal failure typically in the third or fourth decade but with wide variation. It is responsible for about 1% of end-stage renal failure. Over 80% of cases are X-linked and young men are most affected, but heterozygous carriers of the abnormal gene are also at significantly increased risk of end-stage renal failure in their lifetime. Those affected by the autosomal recessive variant are phenotypically very similar. It is caused by mutations in tissue-specific isoforms of basement membrane (type IV) collagen encoded by COL4A5 (X chromosome), COL4A3, and COL4A4 (chromosome 2).
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38

Heidet, Laurence, Bertrand Knebelmann, and Marie Claire Gubler. Alport syndrome. Edited by Neil Turner. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199592548.003.0322_update_001.

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This chapter describes the clinical features of Alport syndrome. The characteristic features of this familial condition are haematuria with progressive nephropathy and sensorineural hearing loss. Most cases are X-linked so this is typically seen in boys and young men, but female heterozygous (‘carriers’) of X-linked Alport syndrome are also at significant risk of renal disease in their lifetime. The average age of end-stage renal failure is in the third or fourth decade. Those with autosomal recessive disease (approximately 15%) show a similar phenotype. Hearing loss characteristically develops during teenage years or as a young adult, usually as proteinuria becomes prominent and renal function begins to be lost. Angiotensin-converting enzyme inhibitors may modify this classic description. Ocular abnormalities are less consistent and tend to occur later, often after end-stage renal failure. Retinal changes do not affect sight. Lenticonus can be treated by lens replacement. Other ocular abnormalities occur rarely. Aortic disease has been reported in occasional families.
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39

Schleiss, Mark R. Cytomegalovirus. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190604813.003.0001.

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Congenital infection with human cytomegalovirus (CMV) is the most common infectious cause of developmental disability in newborns. Congenital CMV is also a leading cause of hearing loss in infants. The overall birth prevalence of congenital CMV is 0.5–1%, varying among different populations. CMV infection may be symptomatic at birth or, more commonly, asymptomatic. Both groups are at risk for sequelae. Antiviral therapy with ganciclovir (or valganciclovir) in infants with symptomatic congenital CMV infection can result in improved neurodevelopmental and hearing outcomes. CMV infections in very low birthweight premature infants, typically acquired via breast milk, can also produce substantial short-term and possibility long-term morbidity. This chapter reviews current concepts regarding the biology of CMV as well as the epidemiology, clinical presentation, diagnosis, management, and outcome of congenital and perinatal CMV infections. High-priority areas for future research, including in the arena of newborn screening for congenital CMV, are discussed.
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40

Clark, Terrell A. Assessment and Development of Deaf Children with Multiple Challenges. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780190880545.003.0002.

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The proportion of children who are deaf or hard of hearing and also have other medical, neurodevelopmental, behavioral, or psychosocial conditions is increasing. Prevalence estimates run as high as 50% to 70%. The shifting complexity challenges not only the learners but also the teachers, administrators, and policymakers responsible for the education of deaf students. Documentation of diagnostic profiles contributes to understanding the learning profile of deaf students with concomitant conditions. This may also inform policy decisions, programmatic design, calibration of parental expectations, and implementation of effective teaching strategies. Through illustrative case examples, this chapter explores the principles of differential diagnosis and the implications of various conditions. Topics covered include genetic syndromes, vestibular dysfunction, intrauterine viral infection with associated congenital hearing loss, autism spectrum disorder, reactive attachment disorder, complex medical histories resulting in severe neurologic compromise, intellectual disabilities, cerebral palsy, and nonsyndromic genetic conditions.
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