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Hereditary deafness in chilldren: Diagnosis and a family report

Published online by Cambridge University Press:  29 June 2007

Reda A. A. Ibrahim  and
Fred H. Linthicum Jr
Reda A. A. Ibrahim
Affiliation:
Lecturer, Department of Ear, Nose, and Throat, Kasr El Einy Hospital, University of Cairo, Egypt; Research Fellow, Ear Research Institute, Los Angeles, California.
Fred H. Linthicum Jr
Affiliation:
Lecturer, Department of Ear, Nose, and Throat, Kasr El Einy Hospital, University of Cairo, Egypt; Research Fellow, Ear Research Institute, Los Angeles, California. 2122 West Third Street, Los Angeles, California, 90057
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Synopsis abstract

A FAMILY with hereditary deafness is presented. The mother and ten of her children suffer sensorineural hearing loss. The maternal grandparents and four uncles and aunts were all hard of hearing. Clinical examination revealed no accompanying gross anomalies. In one child, Mondini deformity is the cause of deafness. In the rest of the family isolated membranous labyrinth deformity is suggested.

The methods of early diagnosis are discussed, among which polytomography proved the most reliable for diagnosing Mondini and Michel deformities. We demonstrated how early fitting of hearing aids is essential for language acquisition and speech development.

Clinical ability to differentiate between subtypes of inner ear hereditary deafness not accompanied by gross anomalies is limited. This problem and the problem arising from late diagnosis are illustrated by a report on a family of ten children, nine of whom suffer sensorineural hearing loss.

Type
Clinical records
Information
The Journal of Laryngology & Otology , Volume 93 , Issue 5 , May 1979 , pp. 495 - 506
Copyright
Copyright © JLO (1984) Limited 1979

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References

REFERENCES

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