Inherited progressive sensorineural hearing loss and
the presence of malformed middle ears. Other features include mental
retardation, narrow palate, and hypogonadism.
Autosomal Dominant, Nonsyndromic Sensorineural Deafness;
Hereditary Low-Frequency Hearing Loss; Konigsmark-Hollander-Berlin Syndrome;
Autosomal dominant. Gene map locus is 5q31.
Short increment sensitivity index (SISI)
examination suggests a cochlear lesion as the source of the hearing loss.
Familial occurrence of low-frequency sensorineural
Sensorineural hearing loss that shows moderate
variation in affected persons. In general, the hearing loss begins in
postlingual (after language and speaking have been acquired),
affecting first the low frequencies and progressing slowly to
involve all frequencies in the latter decades of life.
No specific precautions associated
with this syndrome other than the usual preoperative preparation for an
Anesthetic considerations for any
individual affected with this syndrome are those associated with the
surgical procedures rather than the syndrome itself.
No known specific pharmacological
Konigsmark BW, Mengel MC, Berlin CI: Dominant low-frequency hearing loss:
Report of three families. Laryngoscope
Willems PJ: Genetic causes of hearing loss. N Engl J Med