Abstract
Pendred's syndrome was originally described by Pendred (1896), as the condition of goiter and profound congenital sensorineural deafness. Fraser et al. (1961) reported the findings in 113 individuals from 72 families.
Although the sporadic reports were found in the literature, this condition, particularly from the otological aspect, has not been well described in Japan.
The authors reported two cases in three siblings, and both of them had a similar type of rather severe, symmetrical congenital deafness and goiter that developed at the age of 16 (sister) and 11 (brother). Positive recruitment was found in both cases. As the recent characteristic clinical finding appeared in both cases, vertiginous episodes with nausea, vomiting and fluctuant hearing were occurred occasionally. The abnormal vestibular responses were found in the sister.
