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Objectives: Auditory neuropathy (AN) is a sen-sorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic emissions (OAEs). Many risk factors are thought to be involved in its etiology and patho-physiology. Three Chinese pedigrees with familial AN are presented herein to demonstrate involvement of genetic factors in AN etiology. Methods: Probands of the above - mentioned pedigrees, who had been diagnosed with AN, were evaluated and followed up in the Department of Otolaryngology Head and Neck Surgery, China PLA General Hospital. Their family members were studied and the pedigree diagrams were established. History of illness, physical examination, pure tone audiometry, acoustic reflex, ABRs and transient evoked and distortion - product otoacoustic emissions (TEOAEs and DPOAEs) were obtained from members of these families. DPOAE changes under the influence of contralateral sound stimuli were obs
Objectives: Auditory neuropathy (AN) is a sen-sorineural hearing disorder characterized by absent or abnormal auditory brainstem responses (ABRs) and normal cochlear outer hair cell function as measured by otoacoustic emissions (OAEs). Many risk factors are thought to be involved in its etiology and patho-physiology. Three Chinese pedigrees with familial AN are presented here to demonstrate involvement of genetic factors in AN etiology. Methods: Probands of the above - mentioned pedigrees, who had been diagnosed with AN, were evaluated and followed up in the History of illness, physical examination, pure tone audiometry, acoustic reflex, ABRs and transient evoked and distortion - product otoacoustic emissions ( TEOAEs and DPOAEs were were from members of these families. DPOAE changes under the influence of contralateral soun d stimuli were obs