FREE NEJM E-TOC    HOME   |   SUBSCRIBE   |   CURRENT ISSUE   |   PAST ISSUES   |   COLLECTIONS   |   Search Term  Advanced Search

Sign in | Get NEJM's E-Mail Table of Contents — Free | Subscribe

Previous Volume 342:1101-1109 April 13, 2000 Number 15 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text PDF Purchase this article Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

Hearing loss is the most common sensory defect in humans, affecting normal communication in 10 percent of people aged 65 years or older. In most cases, hearing loss is a multifactorial disorder caused by both genetic and environmental factors. However, single-gene mutations can lead to hearing loss. In these cases, hearing loss is a monogenic disorder with an autosomal dominant, autosomal recessive, X-linked, or mitochondrial mode of inheritance. These monogenic forms of hearing loss can be syndromic (characterized by hearing loss in combination with other abnormalities) or nonsyndromic (with only hearing loss). This review focuses on nonsyndromic hearing loss, since . . . [Full Text of this Article]

Mechanisms of Normal Hearing

Classification of Hearing Loss

Gene-Localization Studies

Genes Implicated in Hearing Loss

POU Transcription-Factor Genes

Myosin Genes

Connexin Genes

KCNQ4 Gene

Diaphanous Gene

Pendrin Gene

-Tectorin Gene

ICERE-1 Gene

COCH Gene

Otoferlin Gene

Mitochondrial Genes

Conclusions

Source Information

From the Department of Clinical Genetics, Erasmus University, Rotterdam, the Netherlands, and the Department of Biochemistry, Physiology, and Genetics, University of Antwerp, Antwerp, Belgium.

Address reprint requests to Dr. Willems at the Department of Clinical Genetics, Erasmus University, Dr. Molewaterplein 50, 3015 GE Rotterdam, the Netherlands.

References

This article has been cited by other articles:

• Gillam, L, Poulakis, Z, Tobin, S, Wake, M (2006). Enhancing the ethical conduct of genetic research: investigating views of parents on including their healthy children in a study on mild hearing loss.. J. Med. Ethics 32: 537-541 [Abstract] [Full Text]  
• Overell, J, Lindahl, A (2004). Neuro-otological syndromes for the neurologist. J. Neurol. Neurosurg. Psychiatry 75: iv53-iv59 [Full Text]  
• Weiss, S., Gottfried, I., Mayrose, I., Khare, S. L., Xiang, M., Dawson, S. J., Avraham, K. B. (2003). The DFNA15 Deafness Mutation Affects POU4F3 Protein Stability, Localization, and Transcriptional Activity. Mol. Cell. Biol. 23: 7957-7964 [Abstract] [Full Text]  
• Jackler, R. K. (2003). A 73-Year-Old Man With Hearing Loss. JAMA 289: 1557-1565 [Full Text]  
• Edmonds, J. L., Kirse, D. J., Kearns, D., Deutsch, R., Spruijt, L., Naviaux, R. K. (2002). The Otolaryngological Manifestations of Mitochondrial Disease and the Risk of Neurodegeneration With Infection. Arch Otolaryngol Head Neck Surg 128: 355-362 [Abstract] [Full Text]  
• Lloyd, R. V., Vidal, S., Jin, L., Zhang, S., Kovacs, K., Horvath, E., Scheithauer, B. W., Boger, E. T. A., Fridell, R. A., Friedman, T. B. (2001). Myosin XVA Expression in the Pituitary and in Other Neuroendocrine Tissues and Tumors. Am. J. Pathol. 159: 1375-1382 [Abstract] [Full Text]  
• Knebelmann, B., Fakhouri, F., Grunfeld, J.-P. (2001). Hereditary nephritis with macrothrombocytopenia: no longer an Alport syndrome variant. Nephrol Dial Transplant 16: 1101-1103 [Full Text]  
• Ben-Yosef, T., Wattenhofer, M., Riazuddin, S., Ahmed, Z. M, Scott, H. S, Kudoh, J., Shibuya, K., Antonarakis, S. E, Bonne-Tamir, B., Radhakrishna, U., Naz, S., Ahmed, Z., Riazuddin, S., Pandya, A., Nance, W. E, Wilcox, E. R, Friedman, T. B, Morell, R. J (2001). Novel mutations of TMPRSS3 in four DFNB8/B10 families segregating congenital autosomal recessive deafness. J. Med. Genet. 38: 396-400 [Full Text]