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February 1990

Temporal Bone Histopathologic Findings in Alagille's Syndrome

Author Affiliations

From the Department of Otolaryngology, University of Pittsburgh (Pa) School of Medicine and Eye and Ear Hospital of Pittsburgh (Drs Okuno, Takahashi, Shibahara, and Sando), and Department of Pathology, University of Pittsburgh School of Medicine and Children's Hospital of Pittsburgh (Dr Hashida). Dr Okuno is now with the Department of Otolaryngology, Faculty of Medicine, University of Tokyo (Japan). Dr Shibahara is now with the Department of Otolaryngology, Tohoku University, Sendai, Japan.

Arch Otolaryngol Head Neck Surg. 1990;116(2):217-220. doi:10.1001/archotol.1990.01870020093025

• Six temporal bones obtained from four individuals with Alagille's syndrome, aged 4 months and 3, 6, and 7 years, were studied histopathologically. The external auditory canals and tympanic membranes were normal. Although the stapes, the interossicular joints, and the subarcuate fossae were slightly underdeveloped in the majority of cases, the other structures in the middle ear were almost normal. However, severe anomalies were observed in structures in the inner ear. In all cases, both the bony and membranous structures of the posterior semicircular canal were partially or totally absent, and, in three ears, those of the anterior semicircular canal were also partially absent; the lateral semicircular canal, however, was normal in all cases. The cochlea was observed to be shortened in only one case.

(Arch Otolaryngol Head Neck Surg. 1990;116:217-220)

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