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.
1
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.
1.1
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 ).
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.
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 |
1.2
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.
2
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 .
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 |