Bilateral osteomas and exostoses of the internal auditory canal




Abstract


Osteomas and exostoses are benign tumors of the bone that occur in the head and neck region but are rarely found within the internal auditory canal (IAC). In this report, we review the literature on bony lesions of the IAC and present two cases: one case of bilateral compressive osteomas and one case of bilateral compressive exostoses of the IAC.



Introduction


Osteomas and exostoses are benign tumors of bone with an estimated prevalence of 0.42% . When present, they are most commonly found in the calvarium, facial bones, and mandible . Rarely, they are found in the internal auditory canal (IAC). Twenty one cases of osteomas and exostoses of the IAC have been reported with only six cases demonstrating bilaterally . Associated symptoms include hearing loss, vestibular dysfunction, tinnitus, and facial spasms. However, patients may be asymptomatic with the lesions only incidentally discovered on radiograph or upon autopsy.


Radiologically, osteomas may be distinguished from exostoses by the presence of bone marrow as well as an acute protrusion into the IAC space. In contrast, exostoses are smooth and broad-based, and are more likely to be bilateral. Definitive distinction between osteomas and exostoses is by histopathology. These secondary causes of IAC stenosis should be distinguished from primary causes such as hyperostotic thickening of the medial temporal bone from pathologies including Paget’s disease, fibrous dysplasia, or osteopetrosis.



Case 1


A 38-year-old woman presented with a one-year history of progressive, bilateral sensorineural hearing loss and tinnitus (L > R), otalgia, and vertigo. High-resolution computed tomography of the temporal bone revealed prominent bony osteomas along the cerebellopontine angle cisterns and porus acusticus bilaterally, right greater than left. The right proximal IAC showed significant stenosis to 2 mm in the AP dimension, and the left IAC showed stenosis to 5 mm ( Fig. 1 ).




Fig. 1


CT axial sections with demonstration of prominent bony osteomas along the cerebellopontine angle cisterns and porus acusticus bilaterally.


Exploration revealed a large osteoma in the jugular tubercle encroaching upon the internal auditory meatus. Because her symptoms were more severe on the left side, microvascular decompression of the left IAC was performed via a retrosigmoidal approach. The osteoma was reduced in a medial to lateral fashion using an ultrasonic surgical aspirator and diamond burrs to decompress and free the vestibulocochlear and facial nerves ( Fig. 1 ).


At follow-up, the patient reported complete resolution of preoperative vertigo, as well as subjective improvement of hearing and tinnitus in her left ear. A post-operative audiogram demonstrated stable discrimination and hearing levels ( Table 1 ). The patient plans to undergo decompression of the right IAC in a staged fashion.



Table 1

Hearing results reported per AAO guidelines.





































Patient Hearing results Pre-treatment PTA Pre-treatment WRS Post-treatment PTA Post-treatment WRS
1 Right ear 92 84 78 84
Left ear 103 76 95 80
2 Right ear 28 88 30 80
Left ear 50 92 23 92



Case 2


An 80-year-old woman presented with sudden asymmetric sensorineural hearing loss (L > R), but denied vertigo, tinnitus, or otalgia. Audiogram showed mild to moderate hearing loss across frequencies. An MRI revealed bilateral bony exostosis of IACs. She was put on a course of prednisone for one month and reported improved hearing on the left side. Repeat audiogram showed significant improvement in the left ear consistent with normal sloping to moderate hearing loss from 250 to 8000 Hz ( Table 1 ). Due to older age and limited symptoms the patient will be serially followed.





Discussion


Our retrospective review of the literature identified 21 unique cases of bony lesions of IAC, including 17 osteomas and 4 exostoses. Three out of 17 cases of osteoma and 3 out of 4 cases of exostoses were bilateral in nature ( Table 2 ) . Symptomatic patients presented at average age of 50 with an average symptom duration of 6.4 years. The most commonly reported symptoms associated with osteomas and exostoses of the IAC have been vertigo (13/21), sensorineural hearing loss (9/21), and tinnitus (9/21) with less common reports of facial paralysis or spasms (2/23). Symptoms are thought to be due to either direct mass compression or by serving as a fulcrum for repetitive trauma with movement . The preponderance of symptoms involving the vestibulocochlear nerve suggests that the facial nerve may be more resistant to compressive effects within the IAC .



Table 2

Reports of bony lesions within the internal auditory canal.














































































































































































































































Author Osteoma or exostosis Gender Age Age at symptom onset Side of lesion Symptoms Treatment Outcome
Baik et al. Osteoma M 70 Right R hearing loss and tinnitus Corticosteroids 2 year follow-up showed no change in lesion; continued tinnitus
Exostosis F 65 47 Bilateral Vertigo Retrosigmoid approach Resolution of vertigo over one year
Exostosis F 55 54 Bilateral Bilateral tinnitus, vertigo
Beale and Phelps Osteoma F 50 49 Right R hearing loss and tinnitus; vertigo None
Ciorba et al. Osteoma M 32 Bilateral No symptoms None
Clerico et al. Osteoma F 59 56 Right Vertigo, tinnitus Suboccipital approach Resolution of vertigo; hearing and balance unchanged
Coakley et al. Osteoma M 42 22 Right Vertigo, tinnitus Retrosigmoid approach Resolution of vertigo and tinnitus
Davis et al. Osteoma F 44 39 Left Episodic vertigo, L facial paralysis Suboccipital approach Significant improvement in symptoms, residual mild vertigo and aural fullness
Osteoma M 51 48 Right Episodic vertigo, R aural fullness Clinical monitoring No change in size after 4.5 years
Doan and Powell Exostosis M 53 53 Right R hearing loss Posterior fossa approach Improvement of R hearing loss
Estrem et al. Osteoma F 56 50 Left Vertigo, L tinnitus Suboccipital approach Resolution of tinnitus, vertigo, motion tolerance, nausea
Gerganov et al. Osteoma F 30 29 Bilateral L hearing loss, vertigo, tinnitus Retrosigmoid approach Resolution of vertigo and tinnitus, slightly improved hearing
Schutt CA et al. Osteoma F 38 37 Bilateral Hearing loss, tinnitus, vertigo Retrosigmoid approach Resolution of vertigo and tinnitus; improvement of L hearing loss
Exostosis F 80 80 Bilateral Hearing loss (L > R) Corticosteroids Improvement of L hearing loss
Liétin et al. Osteoma F 79 49 Left L hemifacial spasms Clinical monitoring
Ramsay and Brackmann Osteoma F 30 26 Left Vertigo, hearing loss, tinnitus Middle fossa approach Resolution of tinnitus and vertigo; improvement of hearing loss
Roberto et al. Osteoma F 33 18 Right Hearing loss, tinnitus, vertigo IAC decompressed Cure of vertigo and tinnitus; hearing loss not improved
Singh et al. Osteoma M 33 31 Right R hearing loss Clinical monitoring
Smelt Exostosis F 67 Bilateral None
Vrabec et al. Osteoma F 31 Bilateral Incidental finding after head injury
Osteoma F 61 59 Left Hearing loss; episodic vertigo Low-salt diet and diuretics 5 year follow-up showed no change in lesion, free of severe vertigo
Osteoma M 59 Left None Clinical monitoring No change in size after 18 months
Wright et al. Osteoma F 34 32 Right Hearing loss, tinnitus Posterior fossa approach 30 dB improvement in pure tone after 6 months

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Aug 23, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on Bilateral osteomas and exostoses of the internal auditory canal

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