Multiple angiomas of the external auditory canal in a patient with Sturge-Weber syndrome.

Sturge-Weber syndrome is a rare, congenital vascular disorder of unknown etiology that affects capillary-sized blood vessels. Facial port-wine stains and associated leptomeningeal angiomas typify the vascular findings. These vascular malformations can cause several neurologic abnormalities, including seizures, mental retardation, hemiparesis, and behavioral disorders. (1)

We report the unusual finding of multiple angiomas of the external auditory canal secondary to Sturge-Weber syndrome. An 11-year-old girl with Sturge-Weber syndrome and associated seizures, mental retardation, behavioral disorder, and hemiparesis was referred to our otolaryngology department for evaluation of conductive hearing loss and recurrent otitis media. Our initial physical examination revealed what appeared to be erythematous, bulging tympanic membranes bilaterally. However, the examination was very limited because of poor patient cooperation, so the patient was anesthetized for microotoscopy. This examination revealed near-total occlusion of both external auditory canals by erythematous, highly vascular masses that arose from the posterior canal walls (figure). These lesions were soft and compressible. When compressed, the visible portion of the tympanic membrane appeared to be normal. Myringotomy detected no fluid in the middle ear.

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Hemangiomas of the external auditory canal occur very rarely. In the cases that have been reported, more men than women were affected, and most cases occurred in the sixth and seventh decades of life. (2)

Resection of the hemangioma is the treatment of choice, and it is usually curative. As in this case, patients may have associated conductive hearing loss. Anecdotal evidence suggests that traditional treatment does not fully restore hearing. (2)

The diagnosis of these vascular malformations of the external auditory canal may be challenging. Physicians must perform a thorough examination and maintain a high level of suspicion to adequately diagnose and manage angiomas of the external auditory canal.

Matthew E. Dahl, MD; Marcella Bothwell, MD; Scot D. Hirschi, MD

References

(1.) Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatr Neurol 2004;30:303-10.

(2.) Limb CJ, Mabrie DC, Carey JP, Minor LB. Hemangioma of the external auditory canal. Otolaryngol Head Neck Surg 2002;126: 74-5.