In 1978 Berlin et al.1 described several severely congenially deaf persons having extraordinarily good speech and better hearing at high and ultra-audiometric frequencies than in the usual speech range. We have identified two families in whom this pattern occurs. Petrous pyramid polytomograms were normal in one family. Two sporadic cases are also described. Inheritance of this audiometric pattern has not been reported previously.
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References
1.
BerlinCIWexlerKFJergerJFHalperinHRSmithS: Superior ultra-audiometric hearing: A new type of hearing loss which correlates highly with unusually good speech in the “profoundly deaf.”Trans Am Acad Ophthalmol Otolaryngol86: 111–116, 1978.
2.
StelmachowiczPGorgaMCullenJ: A calibration procedure for the assessment of thresholds above 8000 Hz. J Speech Hear Res25: 618–623, 1982.
3.
BergstromLThompsonPWoodRPII: New patterns in genetic and congenital otonephropathies. Laryngoscope89: 177–194, 1979.
4.
DeolMS: The development of the inner ear in mice homozygous for shaker-with-syndactylism. J Embryol Exp Morphol11: 493–512, 1963.
5.
SchuknechtHF: Pathology of sensorineural deafness of genetic origin.InMcConnellFWardPH editors: Deafness in childhood. Nashville, 1967, Vanderbilt University Press, pp 69–90.
6.
FujitaSHaydenRC: Alport's syndrome: Temporal bone report. Arch Otolaryngol90: 453–466, 1969.
