OBJECTIVE: AIED (autoimmune inner ear disease) is an autoimmune process that leads to the dysfunction of the inner ear, resulting in fluctuating, audiovestibular symptoms. Although the pathogenesis is likely heterogeneous, immune processes within the inner ear ultimately lead to histopathologic changes and sensorineural hearing loss. This review will discuss the latest evidence on treatment options. METHODS: A literature search on articles pertaining to the treatment of autoimmune inner ear disease was performed on PubMed. RESULTS: Corticosteroid treatment continues to remain as first line therapy for AIED but long-term responsiveness is poor. Cytotoxic chemotherapies can be effective alternatives for steroid nonresponsive patients, but significant side effects may limit their use. Intratympanic steroid injections are beneficial and although there is not enough evidence currently to supplant oral steroid trial they may be a useful adjunct if steroid toxicity is an issue. The efficacy of biologic agents has been variable. Compared to placebo, etanercept does not improve the hearing improvement already attained by steroids alone. However, open pilot studies of other biologic agents show hearing improvements, improvements in tinnitus/aural fullness/vertigo, ability to wean steroid dependency, or benefits in steroid-resistant AIED. CONCLUSION: There is currently not enough evidence that alternative treatments supersede the use of initial steroid treatment. Biologic agents and intratympanic steroid injections are relatively well tolerated and should be considered as adjunctive therapy. More studies on the efficacy of various biologics and more studies on the treatment of steroid resistant disease especially after initial benefit are still needed. For those who eventually lose their hearing, cochlear implantation remains as a viable option. LEVEL OF EVIDENCE: expert opinion.

Purpose of review

This review briefly covers the history of stapedectomy, discusses the indications and problems encountered with revision surgery, and provides case examples with solutions.

Recent findings

Revision surgery is challenging and successful outcome even in the most experienced specialists is 45-71%, which is far less than that of primary surgery.

Summary

Careful evaluation of the reasons for reoperation, anticipation of the common problems, and patient education on reasonable expectations are all very important for success.

Objective

AIED (autoimmune inner ear disease) is an autoimmune process that leads to the dysfunction of the inner ear, resulting in fluctuating, audiovestibular symptoms. Although the pathogenesis is likely heterogeneous, immune processes within the inner ear ultimately lead to histopathologic changes and sensorineural hearing loss. This review will discuss the latest evidence on treatment options.

Methods

A literature search on articles pertaining to the treatment of autoimmune inner ear disease was performed on PubMed.

Results

Corticosteroid treatment continues to remain as first line therapy for AIED but long-term responsiveness is poor. Cytotoxic chemotherapies can be effective alternatives for steroid nonresponsive patients, but significant side effects may limit their use. Intratympanic steroid injections are beneficial and although there is not enough evidence currently to supplant oral steroid trial they may be a useful adjunct if steroid toxicity is an issue. The efficacy of biologic agents has been variable. Compared to placebo, etanercept does not improve the hearing improvement already attained by steroids alone. However, open pilot studies of other biologic agents show hearing improvements, improvements in tinnitus/aural fullness/vertigo, ability to wean steroid dependency, or benefits in steroid-resistant AIED.

Conclusion

There is currently not enough evidence that alternative treatments supersede the use of initial steroid treatment. Biologic agents and intratympanic steroid injections are relatively well tolerated and should be considered as adjunctive therapy. More studies on the efficacy of various biologics and more studies on the treatment of steroid resistant disease especially after initial benefit are still needed. For those who eventually lose their hearing, cochlear implantation remains as a viable option.

Level of evidence

expert opinion.

Tophaceous gout of the middle ear is a rare occurrence that presents as a granular white-colored mass. It is frequently misdiagnosed as cholesteatoma or tympanosclerosis in patients who otherwise may not manifest any clinical or biochemical signs of gout. While uncommon, it can lead to clinically significant disease such as conductive hearing loss. The present report describes 2 cases of middle ear gouty tophi initially mistaken for another entity. Both patients underwent surgery, and the diagnosis of gout was revealed after final histopathological analysis. A review of the literature is also presented.

Objective

We describe three rare cases of spontaneous intracranial hypotension (SIH) presenting with symptoms of endolymphatic hydrops (EH) and perform a literature review to bring attention to a rare link between SIH and EH.

Patient

A 59-year-old female presented with postural headache, aural fullness, vertigo, hearing loss, and abnormal electrocochleography after being diagnosed with SIH by magnetic resonance imaging. The site of cerebrospinal fluid leak was identified in this individual. Two additional patients with vertigo, hearing loss, and SIH were identified by retrospective chart review.

Intervention

All patients underwent blood patches. One patient also had diuretic treatment while another had fibrin glue injection.

Main outcome measures

The outcomes of interest were resolution of headache, vertigo, aural fullness, and hearing loss.

Results

All patients eventually improved with time. Literature review suggests that overall outcome is excellent.

Conclusions

SIH may be an under-recognized cause of EH. We support the theory that negative intracranial pressure transmitted through the cochlear aqueduct and perilymph leads to EH. Despite the variations in treatments, the overall prognosis is excellent.

Objective

This is the first report of multifocal inflammatory pseudotumor (IPT) involving the temporal bone, orbit and paranasal sinus, and the use of rituximab as adjunctive therapy in multifocal temporal bone IPT.

Patient

We describe a 46-year-old man with orbital and maxillary sinus IPT, whose disease progressed despite radiation and steroid burst. He then developed contralateral mastoid disease, otalgia, aural fullness, and hearing loss.

Intervention

He was initiated on rituximab and prednisone therapy. Mastoidectomy with near-total tumor removal was accomplished and histopathology confirmed IPT. A literature review was also performed.

Main outcome measure

Tumor regression or recurrence.

Result

Despite disease progression after radiation and steroids, his orbital, sinus, and mastoid disease improved after surgery, steroids, and rituximab. A review of four other previously reported cases of multifocal disease involving the temporal bone suggest that multifocal disease may be a more aggressive entity with higher recurrence rate compared with solitary disease. Although surgery and steroids are typically recommended, there is currently no consensus treatment recommendation.

Conclusions

Multifocal IPT of the temporal bone is a rare but aggressive entity for which surgery and steroid combination therapy should be first line treatment. We suggest rituximab may be an effective adjunctive treatment particularly for recurrent disease or where systemic therapy may be favored.