Cavitating Lesions around the Cochlea Can Affect Audiometric Threshold and Clinical Practice.

There are several pathologies that can change the anatomy of the otic capsule and that can distort the bone density of the bony structures of the inner ear, but otosclerosis is one of the most frequent. Similar behavior has been shown in patients affected by osteogenesis imperfecta (OI), a genetic disorder due to a mutation in the genes coding for type I (pro) collagen. In particular, we note that otosclerosis and OI can lead to bone resorption creating pericochlear cavitations in contact with the internal auditory canal (IAC). In this regard, we have collected five cases presenting this characteristic; their audiological data and clinical history were analyzed. This feature can be defined as a potential cause of a third-window effect, because it causes an energy loss during the transmission of sound waves from the oval window (OW) away from the basilar membrane.

Cochlear implantation versus auditory brainstem implantation in bilateral total deafness after head trauma: personal experience and review of the literature.

OBJECTIVE: To determine the effectiveness of cochlear implant (CI) in hearing restoration after temporal bone (TB) fractures and investigate the adequacy of auditory brainstem implant (ABI) indication for TB fractures. STUDY DESIGN: Retrospective clinical study; a systematic review of the literature in PubMed was also performed to identify all published cases of bilateral TB fractures or bilateral deafness after head trauma treated by means of CI or ABI. SETTINGS: Quaternary otology and skull base surgery referral center. PATIENTS: Eleven consecutive patients presented with bilateral severe-to-profound sensorineural hearing loss after head trauma. INTERVENTIONS: CI as primary intervention or following a previous treatment. MAIN OUTCOME MEASURES: CI performances were evaluated in the auditory-only condition in both closed-set and open-set formats. RESULTS: Fourteen CI were placed, 11 as primary treatment and 3 after ABI failure. At the last follow-up, all patients gained useful open-set speech perception. In secondary CI, all patients obtained better auditory results with the CI if compared with ABI. CI performance did not decrease with time in any case. CONCLUSION: Cochlear implantation after TB fractures has proved to have excellent audiometric results. The aim of the initial evaluation of a patient with bilateral anacoustic ears from head trauma should always be to rehabilitate their hearing with a CI. The incidence of labyrinthitis ossificans, negative electrophysiologic testing, the risk of postoperative meningitis or facial nerve stimulation should not be the determinant factors that favor ABI placement.

Indications and contraindications of auditory brainstem implants: systematic review and illustrative cases.

The number of non-neurofibromatosis type 2 (NF2) indications for auditory brainstem implant (ABI) in the literature is increasing. The objective of this study was to analyze and discuss the indications for ABI. Retrospective chart review and systematic review were conducted at Quaternary referral skull base center and referring centers. Analysis of ABI cases with non-NF2 indications and systematic review presenting non-NF2 ABI cases were performed. Fourteen referred cases with ABI were identified. All cases had unsatisfactory results of ABI and all could have been rehabilitated with a cochlear implant (CI). Of these 14 cases, 9 improved with a cochlear implant, and 2 with a hearing aid, two are still planned for CI, one received bilateral CI, no ABI. In literature, we found 31 articles presenting 144 non-NF2 ABI cases with at least 7 different indications other than NF2. ABI should be restricted to those patients who have no other rehabilitation options. Patency of the cochlea and evidence of an intact cochlear nerve should be examined with imaging and electrophysiologic testing. Sometimes a CI trial should be planned prior to proceeding with ABI. We have shown that in many cases a CI is still possible and CI provided better results than ABI. In vestibular schwannoma in the only hearing ear, cochlear otosclerosis, temporal bone fractures, (presumed) bilateral traumatic cochlear nerve disruption, auto-immune inner ear disease and auditory neuropathy primarily CI are indicated. Traumatic bilateral cochlear nerve disruption is exceptionally unlikely. In cochlear nerve aplasia, testing should be performed prior to meeting indications for ABI. In malformations, ABI is indicated only in severe cochlear hypoplasia or cochlear aplasia.

Preoperative protective stenting of the internal carotid artery in the management of complex head and neck paragangliomas: long-term results.

OBJECTIVES: To investigate the long-term results of preoperative stenting of the internal carotid artery (ICA) in complex head and neck paragangliomas (HNP) as well as to report on indications and technical details of the procedure. METHOD: A comprehensive retrospective review of patients affected by HNP, consecutively operated on and preoperatively treated with stenting of the ICA in a quaternary referral skull base center, was performed. RESULTS: Nineteen patients affected by complex HNP were identified, on whom 21 preoperative stenting procedures were performed. The mean follow-up period after stent insertion was 53.8 months; the patients' age ranged from 33 to 56 years. Fourteen patients were affected by tympanojugular paragangliomas, 4 by vagal paragangliomas and 1 by bilateral carotid body tumors. Five patients presented with recurrent tumors, while 7 presented with multiple or bilateral HNP. There were no complications associated with endovascular procedures. Total tumor removal was accomplished in 52.4% of the cases with 1 recurrence. An advanced stage was the main factor conditioning total removal. Clinical control was obtained in 80% of the patients with residual disease. Total tumor removal from and around the ICA was obtained in 95.2% of the cases. Long-term stent patency was evident in 20 of 21 cases. CONCLUSIONS: Preoperative stenting of the ICA represents a safe and effective procedure in selected cases, obviating the need for balloon occlusion or bypass procedures and reducing the risk of intraoperative vascular injury.

Current trends in the management of the complications of chronic otitis media with cholesteatoma.

PURPOSE OF REVIEW: Complications of cholesteatoma can be of a different nature from those of other otitis media. This review aims to undertake an analysis of current literature regarding management of the complications of cholesteatoma. RECENT FINDINGS: Despite a significant decline in the incidence of complications secondary to cholesteatoma in developed countries it is still a considerable problem in the developing countries. Among intratemporal complications, facial nerve paralysis and labrynthine fistula and among intracranial complications, meningitis, brain abscess and lateral sinus thrombosis are most common. In cases of facial nerve paralysis, decompression with complete disease eradication is considered to be the mainstay of treatment and usefulness of an epineural incision and the range of the decompression are still debatable. Labyrinthine fistula is commonly managed by a single staged matrix removal, followed by closure of the fistula. Partial labrynthectomy in difficult cases is gaining favor among surgeons today. Meningitis and brain abscesses are treated with antibiotics and steroid therapy followed by surgery when the patient is neurologically stable. In lateral sinus thrombosis, mastoidectomy and removal of infected tissue is the primary treatment. Sinus incision and thrombectomy does not seem to improve recanalization and anticoagulation is usually not necessary. Treatment of meningoencephalic herniations is based mainly on the diameter of the herniation. SUMMARY: There is considerable debate in the management of almost every complication of cholesteatoma. Multicentric studies to compare the efficacies of various treatment modalities are the need of the hour to come to definitive conclusions regarding the best treatment options.

The role of subtotal petrosectomy in cochlear implant surgery--a report of 32 cases and review on indications.

OBJECTIVE: To report and review 32 cases of subtotal petrosectomy (SP) in cochlear implant (CI) surgery and to define the indications and contraindications for this procedure. STUDY DESIGN: Retrospective case review + case reports. SETTING: Tertiary skull base center. PATIENTS: Cochlear implant database: 32 subtotal petrosectomies in 31 patients. INTERVENTIONS: Subtotal petrosectomy with blind sac closure of the external auditory canal, closure of Eustachian tube, and abdominal fat obliteration in combination with cochlear implantation. RESULTS: Indications for SP in CI surgery were as follows: chronic otitis media (n = 4), previous radical cavity (n = 13), previous subtotal petrosectomy (n = 4), ossification of the cochlea (n = 5), malformation of the inner ear (n = 2), and temporal bone fracture (n = 4). One patient was simultaneously bilaterally implanted; 2 cases were revisions. All procedures were performed in 1 stage. In 2 cases, complications were encountered (6%), one of which lead to reoperation (3%). None of the patients was explanted. CONCLUSION: Subtotal petrosectomy combined with cochlear implantation is a procedure required in specific situations and lowers the risk of repetitive ear infections, CSF leakage, and meningitis by closing off all connection with the external environment. Additionally, it gives excellent visibility and access in difficult anatomy or in drill-out procedures. The complication rate of 6% is comparable with normal cochlear implantation. Preservation of residual hearing can be considered the only absolute contraindication as an open external meatus is necessary for use of electroacoustic stimulation. Risks of the SP+CI procedure are infection of the abdominal fat, breakdown of the blind sac closure, and entrapped cholesteatoma. Follow-up with CT imaging is therefore mandatory.

Vestibular schwannoma in the only hearing ear: role of cochlear implants.

OBJECTIVES: We sought to delineate the role of cochlear implantation in the management of vestibular schwannoma or other cerebellopontine angle tumors in the only hearing ear. METHODS: We performed a retrospective analysis in a quaternary referral skull base center of all patients who were affected by vestibular schwannoma (or other lesions of the cerebellopontine angle) in the only hearing ear and received a cochlear implant before or after tumor treatment (surgery or radiotherapy) or during the wait-and-scan follow-up. We also performed a systematic review of the English-language literature. RESULTS: The clinical and audiological results of 10 patients are reported. All patients were managed with contralateral cochlear implantation. In 7 patients, cochlear implantation was performed before tumor removal, while hearing in the ear with the tumor was still present. In 3 patients, the implant was placed after curative surgery. Nine of the 10 patients routinely use their implant with subjective benefit and fairly good auditory performance (median disyllabic word recognition, 90%; median sentence comprehension, 75%). The literature search retrieved no major series with assessment of the long-term efficacy of cochlear implantation in this rare clinical scenario. CONCLUSIONS: Patients affected by vestibular schwannoma in their only hearing ear may significantly benefit from a cochlear implant on the contralateral side prior to tumor removal. Recent and significant hearing deterioration and tumor growth represent the main indications for cochlear implantation.

Head and neck paragangliomas: genetic spectrum and clinical variability in 79 consecutive patients.

Head and neck paragangliomas (HNPGLs) are neural crest-derived tumors. In comparison with paragangliomas located in the abdomen and the chest, which are generally catecholamine secreting (sPGLs) and sympathetic in origin, HNPGLs are, in fact, parasympathetic in origin and are generally nonsecreting. Overall, 79 consecutive patients with HNPGL were examined for mutations in SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, MAX, and TMEM127 genes by PCR/sequencing. According to a detailed family history (FH) and clinical, laboratory (including metanephrines), and instrumental examinations, patients were divided into three groups: a) patients with a positive FH for HNPGL (index cases only), b) patients with a negative FH and multiple HNPGLs (synchronous or metachronous) or HNPGL associated with an sPGL, and c) patients with negative FH and single HNPGL. The ten patients in group a) proved to be SDHD mutation carriers. The 16 patients in group b) proved to be SDHD mutation carriers. Among the 53 patients in group c), ten presented with germ-line mutations (three SDHB, three SDHD, two VHL, and two SDHAF2). An sPGL was found at diagnosis or followed up in five patients (6.3%), all were SDHD mutation carriers. No SDHC, SDHA, MAX, and TMEM127 mutations were found. In SDHD mutation carriers, none of the patients affected by HNPGL associated with sPGL presented missense mutations. In conclusion, a positive FH or the presence of multiple HNPGLs is a strong predictor for germ-line mutations, which are also present in 18.8% of patients carefully classified as sporadic. The most frequently mutated gene so far is SDHD but others, including SDHB, SDHAF2, and VHL, may also be affected.

Enlarged translabyrinthine approach with transapical extension in the management of giant vestibular schwannomas: personal experience and review of literature.

OBJECTIVE: To describe and analyze the main outcomes achieved in a series of patients with sporadic vestibular schwannoma (VS) larger than 40 mm in extrameatal diameter, defined as giant VS, submitted to microsurgery by the enlarged translabyrinthine approach with transapical extension. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: A retrospective chart review was conducted on 2,133 patients who underwent surgery for VS from April 1987 to July 2009. One hundred ten cases of giant VS were elected for analysis. MAIN OUTCOME MEASURES: Extent of removal, residual or recurrent disease, facial nerve integrity during surgery, long-term facial nerve function, and postoperative complications. RESULTS: Total removal was accomplished in 91.8% of cases. In 5 patients (4.5%), total removal was accomplished in 2 stages. Near-total removal was performed in 7 patients (6.3%). The facial nerve was anatomically preserved in 76.4% of cases. Intraoperative facial nerve reconstruction was performed in 8 cases. Facial nerve function after 1 year of follow-up was House-Brackmann grades I to III in 75% of cases. There were no deaths in this series. Neurovascular life-threatening complications occurred in 2 patients. Cerebrospinal fluid leak was present in 1.8% of cases. CONCLUSION: Results indicate the enlarged translabyrinthine approach with transapical extension as an elective approach for removal of giant VS. The method permits achievement of a high rate of total removal with low incidence of complications.

Clinically guided genetic screening in a large cohort of italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas.

PURPOSE: The aim of the study was to define the frequency of hereditary forms and the genotype/phenotype correlations in a large cohort of Italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas. DESIGN: We examined 501 consecutive patients with pheochromocytomas and/or paragangliomas (secreting or nonsecreting). Complete medical and family histories, as well as the results of clinical, laboratory, and imaging studies, were recorded in a database. Patients were divided into different groups according to their family history, the presence of lesions outside adrenals/paraganglia considered syndromic for VHL disease, MEN2, and NF1, and the number and types of pheochromocytomas and/or paragangliomas. Germ-line mutations in known susceptibility genes were investigated by gene sequencing (VHL, RET, SDHB, SDHC, SDHD) or diagnosed according to phenotype (NF1). In 160 patients younger than 50 yr with a wild-type profile, multiplex ligation-dependent probe amplification assays were performed to detect genomic rearrangements. RESULTS: Germline mutations were detected in 32.1% of cases, but frequencies varied widely depending on the classification criteria and ranged from 100% in patients with associated syndromic lesions to 11.6% in patients with a single tumor and a negative family history. The types and number of pheochromocytomas/paragangliomas as well as age at presentation and malignancy suggest which gene should be screened first. Genomic rearrangements were found in two of 160 patients (1.2%). CONCLUSIONS: The frequency of the hereditary forms of pheochromocytoma/paraganglioma varies depending on the family history and the clinical presentation. A positive family history and an accurate clinical evaluation of patients are strong indicators of which genes should be screened first.

Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation.

OBJECTIVE: We aimed to evaluate and compare the auditory performance of neurofibromatosis type 2 (NF2) patients with bilateral total deafness fitted with cochlear or auditory brainstem implants. PATIENTS AND METHODS: A retrospective case review was performed. Nine patients suffering from NF2 who underwent hearing rehabilitation by means of cochlear (4 patients) or auditory brainstem (5 patients) implantation participated in the study. Postoperative auditory performance was assessed using closed- and open-set tests. RESULTS: In the group of patients fitted with a cochlear implant, 3 subjects achieved open-set speech recognition abilities comparable to those of standard adult postlingual implant patients; the remaining patient scored 0% in all open-set format tests, reporting benefits only in environmental sound detection and lip-reading. Among the 5 patients who underwent auditory brainstem implantation, 1 reached good open-set speech recognition skills, scoring 70% in the common phrases comprehension test, and she was able to communicate on the telephone. Two other patients achieved open-set speech understanding (respectively, 33 and 41% in the common phrases comprehension test), reporting daily use of their device. The remaining 2 patients did not achieve any level of open-set speech perception, obtaining only improved access to environmental sound and lip-reading skills. CONCLUSIONS: Our study confirmed literature data reporting that cochlear implantation may offer open-set speech communication in NF2 patients. In this small cohort, cochlear implant patients performed better than auditory brainstem implant patients, even if variability in auditory performance was observed with both devices. More studies are needed in order to clarify the role and reliability of electrophysiological tests in predicting the residual functionality of the cochlear nerve after tumor removal.

Inner ear extension of vestibular schwannomas.

OBJECTIVE: Inner ear extension of vestibular schwannomas (VSs) is a rare finding but has important clinical implications. This report reviews the treatment options and presents the experience of the Gruppo Otologico, Piacenza, Italy, in this field. STUDY DESIGN: Case report and literature review. METHODS: Five cases of VSs with inner ear extension were surgically removed. In all of them, the cochlea was partially or completely invaded by the lesion. RESULTS: In 4 cases, the inner ear extension was preoperatively identified on magnetic resonance imaging, and the surgical removal was planned through a transotic approach. In the last case, the cochlear invasion was not detected preoperatively, and the lesion was removed during a second surgery performed to seal a cerebrospinal fluid fistula. CONCLUSIONS: VSs with inner ear extension should be distinguished from pure intralabyrinthine schwannomas because of differences in clinical significance. Cochlear involvement is more frequent than vestibular involvement and is often accompanied by a dead ear. Dead ear caused by small VSs should alert the surgeon to the possibility of a cochlear extension. The presence of an intracochlear involvement requires the adoption of an approach that allows control of the cochlear turns, and we found the transotic approach to be the most suitable. Undetected cochlear extensions that are left in place may grow with time.