Sudden hearing loss with simultaneous posterior semicircular canal BPPV: Possible etiology and clinical implications

Objectives

The objectives of this study were to describe the clinical course and outcome of patients with sudden sensorineural hearing loss (SSNHL) in conjunction with benign paroxysmal positional vertigo (BPPV), and hypothesize the possible pathophysiology of this entity.

Study design

Retrospective study of all patients with evidence of SSNHL with any type of BPPV between 2008 and 2012.

Settings

Tertiary care university hospital.

Subjects and methods

Five patients aged 56 to 71 were diagnosed with unilateral profound SSNHL and BPPV. Neurotologic examination revealed an ipsilateral torsional, up-beating nystagmus on Dix–Hallpike exam. Severe or profound ipsilateral–sensorineural hearing loss was recognized on audiometry. The rest of the exam was normal; this was in keeping with the diagnosis of SSNHL with ipsilateral posterior semicircular canal BPPV.

Results

All patients were treated with a modified Epley maneuver; oral steroids were administered for two weeks. In all cases vertigo resolved and the Dix–Hallpike exam became normal within several weeks. However, the hearing loss remained unchanged in two patients. Magnetic resonance imaging of the head was normal and ENG caloric test demonstrated mild ipsilateral canal paresis in two patients.

Conclusions

1. Patients with SSNHL and BPPV can have a variable clinical course and outcome. This entity may be quite common, but the diagnosis of BPPV can be missed if a complete neurological physical examination is not performed. 2. Arterial occlusions or selective multiple vascular or neural involvement may explain the pathophysiology of SSNHL with BPPV of the posterior semicircular canal.     

Bilateral secondary neurolymphomatosis of the internal auditory canal nerves: A case report

Background

Neurolymphomatosis describes the malignant lymphomatous infiltration of nerves.

Methods

We encountered a unique case of a 47-year-old patient with non-Hodgkin's lymphoma presenting with bilateral sensorineural hearing loss, vestibular dysfunction and bilateral facial nerve palsy.

Results

Magnetic resonance imaging demonstrated enhancement and thickening of internal auditory canal nerves bilaterally consistent with neurolymphomatosis. Patient was treated with combined intrathecal chemotherapy and total brain irradiation.

Conclusions

One must always remain vigilant for metastatic disease in patients with sensorineural hearing loss and/or vestibular dysfunction and facial nerve palsy in the context of known malignancy.     

The limitations of computed tomography in adult cochlear implant evaluation

Objective

To demonstrate the added value of magnetic resonance imaging (MRI) over computed tomography (CT) during adult cochlear implant evaluation.

Patients

Two adult patients are discussed in whom MRI studies diagnosed bilateral vestibular schwannomas during cochlear implant candidacy evaluation.

Interventions

Temporal bone CT and MRI.

Main outcome measure

Diagnosis of NF2.

Results

Two adult patients, ages 67 and 68 years, were evaluated for cochlear implant candidacy. Both patients experienced slowly progressive, bilateral hearing loss without complaints of vertigo, and neither patient had a family history of hearing loss or neurogenic tumors. Both patients had near-symmetric pure tone thresholds on audiometric testing. An MRI and a CT scan were performed on both patients, and bilateral vestibular schwannomas were identified on MRI.

Conclusions

Though MRI is not routinely utilized in adult cochlear implant evaluation, it may be of greater clinical value than CT in the setting of adult-onset hearing loss. MRI allows for sensitive evaluation of cochlear patency and architecture, and cochlear nerve status. As demonstrated in the two index cases, MRI also provides the added benefit of evaluating for causes of retrocochlear hearing loss. These two patients would have likely experienced a significant delay in diagnosis of NF2 without preoperative MRI, particularly given the limitations of scanning following CI magnet placement.     

Cochlear implantation in 13q deletion syndrome

Purpose

The advent of cochlear implantation has been followed by an explosion in surgical and technological advances with subsequent alterations in the treatment of sensorineural hearing loss management. Many individuals with rare genetic abnormalities who once may have not been considered candidates for cochlear implantation are now benefiting from cochlear implantation. One of these unusual syndromes is the 13q deletion syndrome. The clinical features of 13q deletion syndrome, a rare chromosomal abnormality, were originally described in the early 1960s, though the literature currently lacks defined phenotypic abnormalities. Patients with 13q deletion syndrome present with varying degrees of cognitive and growth delay, craniofacial dysmorphisms, and congenital malformations. The etiology of the sensorineural hearing loss has not been elucidated, and genes associated with other syndromes which include sensorineural hearing loss have been proposed as the causative agents. We describe the unique clinical and surgical considerations for 13q deletion syndrome and review the considerations when deliberating on cochlear implantation in syndromic hearing loss.

Materials and Methods

Case report.

Results

Successful cochlear implantation in a patient with 13q deletion syndrome.

Conclusion

Many patients with 13q deletion syndrome suffer from sensorineural hearing loss, and when appropriately selected may likely benefit from cochlear implantation. Many other syndromic patients following careful selection may also benefit from cochlear implantation and should not be excluded from consideration until appropriately evaluated.     

Magnetic resonance imaging-detected inner ear hemorrhage as a potential cause of sudden sensorineural hearing loss

Purpose

The aim of this study is to assess the value of magnetic resonance imaging in identifying the etiology of sudden sensorineural hearing loss, and to correlate the high signals in the labyrinth with clinical features to identify if inner ear hemorrhage could be implicated.

Materials and methods

In this retrospective study, inner ear magnetic resonance imaging was given to 112 patients with sudden sensorineural hearing loss in the First Affiliated Hospital of Sun Yat-sen University from 2011 to 2012. The clinical features of patients with high signals in the labyrinth on magnetic resonance imaging were analyzed.

Results

Abnormal magnetic resonance images were identified in 13 (11.6%) patients. Retrocochlear pathology was found in six patients, including two cases of lacunar infarction, one case of multiple ischemias in the brainstem and bilateral centrum semiovale, two cases of acoustic neuroma, and one case of inner ear hemangioma. There were seven cases showing high signals in the labyrinth on unenhanced T1-weighted and fluid-attenuated inversion recovery images. Clinical features of these seven patients were characterized by irreversible profound hearing impairment and vestibular dysfunction. These findings were consistent with the hypothesis that their symptoms were caused by an inner ear hemorrhage.

Conclusion

The results indicate the importance of magnetic resonance imaging in sudden sensorineural hearing loss in patients. Moreover, patients with vestibular dysfunction and sudden profound hearing loss may have an inner ear hemorrhage evident by interpreting clinical and magnetic resonance imaging results.     

Simultaneous translabyrinthine removal of acoustic neuroma and cochlear implantation

Objectives

To report of a 65-year-old woman with bilateral Meniere's disease was referred for cochlear implantation (CI) due to severe/profound sensorineural hearing loss.

Methods

During the assessment workup, a vestibular schwannoma in the right ear was found by MR imaging. She underwent a translabyrinthine removal of the acoustic neuroma (AN) with sparing of the cochlear nerve and concurrent ipsilateral CI with a Nucleus Freedom device (Cochlear Ltd., Lane Cove, New South Wales, Australia).

Results

Complete removal of the AN was achieved without complications. Neural Response Telemetry (NRT) measurements, which showed poor morphology at the intraoperative tests, rapidly improved after activation, similarly to electrically evoked auditory brainstem responses (E-ABR). The patient reached 100% speech perception performances within 2 months from implantation, in the monaural condition. She was relieved from vertigo spell up to 14 months after the operation.

Conclusion

Cochlear implantation at the time of acoustic neuroma removal with VIII nerve sparing can be a safe and effective hearing restoration procedure.     

Outcomes of Severe to Profound Idiopathic Sudden Sensorineural Hearing Loss

Objectives

While a severe to profound sudden sensorineural hearing loss (SSNHL) may cause serious disability in verbal communication, there have been little studies focusing on this high degree SSNHL. The present study was aimed to investigate the characteristics of hearing recovery in a high degree SSNHL (>70 dB).

Methods

Three hundred and two SSNHL patients were enrolled. For a long-term follow-up, 46 patients were evaluated. Hearing level was examined by pure tone audiometry on day 1, week 3, month 3, month 6, and year 1 or after. According to the degree of the initial hearing loss, the patients were divided into 4 groups from 70 to ≥100 dB.

Results

After 3 weeks, the recovery rate and mean hearing gain was 61%, 23.85 dB in the 70 dB group, whereas 10%, 6.61 dB in the ≥100 dB group. There was a significant correlation between 3-week recovery and final hearing outcome. However, there was almost no recovery after 3 months.

Conclusion

An early recovery can be a prognostic factor for the final recovery in severe to profound SSNHL. Since recovery after 3 months is rare, an early hearing intervention like hearing aid or cochlear implantation should be considered in the high degree SSNHL to restore the patient''s verbal communication.     

Evaluation of cochlear involvement by distortion product otoacoustic emission in Behçet's disease

Objective

The aim of this study was to investigate cochlear involvement in patients with Behçet's disease.

Materials and methods

Twenty-six Behçet's disease patients (52 ears) and 24 sex and age-matched healthy control subjects (48 ears) were included in the study. Pure-tone audiometry at frequencies 250, 500, 1000, 2000, 4000, 6000 Hz, immittance measures including tympanometry and acoustic reflex testing and DPOAE (distortion product otoacoustic emission) testing were performed in the patients and controls.

Results

A sensorineural hearing loss was found in eight patients (30.7%) as it was bilateral in five and unilateral in three patients. Although no typical audiometric configuration was found, one patient had a flat type audiogram, and the others had a high frequency hearing loss. The DPOAE responses of the patients and controls were significantly different in all frequencies (p < 0.05).

Conclusion

These results indicate that cochlea is affected by damage of outer hair cells in Behçet's disease.     

Residual tinnitus after the medical treatment of sudden deafness

Objectives

Some patients with sudden sensorineural hearing loss (SSNHL) are frustrated by residual tinnitus even after accomplishment of the treatment for SSNHL. In the present prospective study, we examined patients’ backgrounds of sex, laterality and age together with changes in hearing level and the tinnitus score after the onset of SSHNL to determine the prognostic factors of residual tinnitus after the final day of medical treatment for SSNHL.

Methods

Forty-four patients with SSNHL were all treated with systemic administration of steroids for 2 weeks and oral intake of vasoactive drugs and vitamin B12 for 6 months before accomplishment of the treatment for SSNHL. The hearing improvement rate (HIR) was determined by comparing the hearing level before and 6 months after the start of treatment. Tinnitus was subjectively evaluated by the tinnitus scoring questionnaire before, 6 and 24 months after the start of treatment. The score of a five-step evaluation of subjective tinnitus feelings, “loudness”, “duration” and “annoyance”, was recorded.

Results

HIR was significantly correlated with tinnitus score improvement (TSI) in “duration” at 6 months after the start of treatment compared with before treatment. The tinnitus score of all 3 items was significantly improved 6 months after the start of treatment compared with that before treatment but it was not significantly changed between 6 and 24 months after the start of treatment. TSI in “duration” between 6 and 24 months was significantly correlated with the patients’ age and HIR using multiple regression analysis.

Conclusion

According to the tinnitus scoring questionnaire, “duration” is the most reliable item for subjective evaluation of tinnitus accompanied by SSNHL. Generally, subjective feelings for residual tinnitus 6 months after the start of treatment for SSNHL are supposed to be almost the same, even at the 24th post-treatment month. Especially, younger patients with better hearing improvement are predicted to achieve further improvement of tinnitus between 6 and 24 months after the start of treatment.     

Epicutaneous patch test—A new diagnostic option to prevent the rejection of silicone-covered cochlear implants in children

Objectives

Cochlear implantation (CI) is a revolutionary method for hearing rehabilitation in patients with severe or profound sensorineural hearing loss. One of the surgical complications may be the necrosis of the skin flap above the receiver-stimulator coil, resulting in device extrusion. Our aim was to find the plausible causes of the silicone covered implant rejection.

Patients and methods

Authors present four cases of cochlear implant rejection, briefly describe their dermato-surgical solutions and analyse their innovative method – the epicutanoeus patch testing with silicone samples.

Results

They observed positive skin reaction in three of the four cases.

Conclusion

Authors analyse the applicability and results of their surgical solutions and the epicutaneous testing in connection with the prevention of skin flap necrosis and rejection of silicone-covered cochlear implants.     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

Paediatric cochlear implantation in north-western Nigeria case report and review of our challenges

Objective

To report the commencement of cochlear implantation (CI) in children in Northern Nigeria and review the challenges encountered with a view to establishing CI programme.

Cases

Two children with post-meningitic sensorineural hearing loss had standard pre-implantation assessment and cochlear implantation surgery in Nigeria in conjunction with surgeons from another developing country. Post-implantation meningitis was encountered and managed in one of the patients. Post-CI rehabilitation started in a foreign country and continued in our centre after training of our speech therapist. Both patients had improvement in their hearing.

Conclusion

These showcase successful CI in patients from Sub-Saharan Africa, the challenges included need for development of surgical and post-CI rehabilitation personnel and affordability of the cost of the implant. The support of all the stakeholders needed for commencement of a CI programme was noted.     

Is BPPV a Prognostic Factor in Idiopathic Sudden Sensory Hearing Loss?

Objectives

The prognostic significance of vertigo in patients with idiopathic sudden sensorineural hearing loss (SSNHL) remains a matter of debate because vertigo is associated with many different vestibular disorders. The purpose of this study is to determine the role of benign paroxysmal positional vertigo (BPPV) as a prognostic factor in patients with SSNHL.

Methods

We conducted a retrospective study of 298 patients with SSNHL. Hearing outcomes were evaluated by assessments of pre-treatment hearing and hearing gain. Comparative multivariate analyses between prognostic factors and hearing outcome were conducted.

Results

Thirty-eight (12.7%) SSNHL patients were found to also have BPPV. BPPV showed significant negative prognostic factors in hearing outcome on multivariate analysis (odds ratio, 0.15). In comparison to average pure tone audiometry (PTA), patients diagnosed with SSNHL with BPPV exhibited poorer hearing in pre- and post-treatment PTA compared to SSNHL without BPPV. Old age (>60 years), pre-treatment hearing, and canal paresis were significant outcome predictors.

Conclusion

BPPV in SSNHL patients, representing definitive vestibular damage, was closely related to poor prognosis.     

Otolaryngological presentations of Cornelia de Lange syndrome

Aim

Children with Cornelia de Lange syndrome frequently present to otolaryngology services with hearing problems. Airway problems have not previously been reported. We wish to describe our experience of the overall management in a series of children with Cornelia de Lange syndrome.

Methods

Retrospective case note review of children diagnosed with Cornelia de Lange syndrome presenting to our department between 2005 and 2014.

Results

Six patients were seen. Airway problems consisted of laryngeal overspill with severe gastroesophageal dysmotility and reflux despite structurally normal airway (1 case), laryngomalacia requiring supraglottoplasty (2 cases), reflux laryngitis with secondary laryngomalacia and coincidental tracheal diverticulum (1 case) choanal atresia requiring stents (1 case) and obstructive sleep apnoea (1 case). Supraglottoplasty produced a dramatic improvement in feeding and breathing in both children who underwent the procedure. Two children had palatal anomalies and one underwent cochlear implantation for a profound sensorineural hearing loss.

Conclusion

Children with Cornelia de Lange syndrome have multifaceted ENT problems. Airway pathology has not previously been described in Cornelia de Lange syndrome but has been common in our experience. We wish to highlight that laryngomalacia in Cornelia de Lange syndrome responds well to supraglottoplasty.     

Acquisition of early auditory milestones with a cochlear implant

Objective

Speech acquisition after cochlear implant is a long process. Various studies have followed the auditory milestones in the early period after implantation. The aim of the present study was to track the development of hearing skills in the early period after cochlear implantation and evaluate which factors influence the process.

Methods

195 records of children implanted in the Hadassah Medical Center were examined retrospectively. Data on etiology, age at implantation and type of implant were collected. In addition, information on the rate of progress was measured: the first time that there was detection and identification of Ling sounds, the first time it was possible to obtain SDT (speech detection threshold), SRT (speech reception threshold) and an audiogram, and the first accurate repetition of VCV (vowel consonant vowel) sounds.

Results

Results show a consistent pattern of auditory milestone acquisition similar to that of normal development, from milestones that do not require decoding beginning with SDT, detection of Ling sounds followed by an audiogram which requires cooperation, to tasks that involve decoding starting with SRT and repetition of Ling sounds and finally VCV repetition.The children implanted before 24 months of age achieved the auditory milestones later than children implanted between 2 and 6 years, apparently since these tasks involve cognitive abilities which are not yet developed in the youngest children.Previous hearing experience improved the rate of acquisition of the auditory milestones and progress was faster in the second implanted ear compared to the first implanted ear.

Conclusion

More research is needed to address the relationship between acquisition of early auditory milestones and performance with the cochlear implant later on in life.     

Considerations on the physiopathological mechanism of inner ear damage induced by intravenous cocaine abuse: cues from a case report

Objective

Aim of the following paper is to discuss about the possible etiopathogenetic mechanisms of inner ear damage induced by cocaine abuse. Unfortunately the data concerning this topic are very limited; the authors are then presenting a literature review, also discussing the clinical presentation and the possible therapeutical approach of a clinical case of bilateral sudden sensorineural hearing loss following i.v. injection of cocaine.

Patients

Case report.

Intervention

A strictly audiological evaluation has been performed, in order to identify the cochlear lesion site(s) and to provide the best medical treatment.

Conclusions

To our knowledge, this is the first report of acute cocaine intoxication with sudden bilateral hearing loss. Further studies are required in order to understand the effects of these substances on the inner ear cells and metabolism.     

Magnetic resonance imaging and bone anchored hearing implants: Pediatric considerations

Objective

Recent developments in bone conduction hearing systems have seen the introduction of transcutaneous devices comprising of magnetic components. Our aim was to identify the number of children implanted with a traditional, non-magnetic percutaneous bone anchored hearing implant (BAHI) who would not have been eligible for a transcutaneous implant based on magnetic resonance imaging (MRI) need.

Methods

A retrospective case review of 206 children who had a percutaneous BAHI at the Birmingham Children's Hospital (January 2009–October 2012) for auditory rehabilitation.

Results

Twenty-eight percent (56/206) of children required at least one MRI scan after receiving a BAHI and 10 percent of patients (20/206) required two or more MRI scans. The main indication for MRI scanning was for neurological co-morbidities; a MRI brain was the most common scan performed.

Conclusion

Although transcutaneous hearing devices/middle ear implants have their clear benefits, it may be argued that these relatively more invasive surgical procedures may not be the best option for the child who will require MRI scanning at some point in the future. Clinicians should be mindful of any need for MRI scanning when considering implant choices in the pediatric population.     

Middle ear metastasis of thymoma

Objective

We report an extremely rare case of thymoma which developed middle ear metastasis along with acute sensorineural hearing loss in the contra ear.

Method

We present a case report and a review of the world literature concerning thymoma metastases to the middle ear.

Results

A 54-year-old female patient with thymoma who developed middle ear metastasis along with acute sensorineural hearing loss in the contra ear.

Conclusion

We have not found out thymoma metastases to the middle ear in the past.     

Cochlear implants in children deafened by congenital cytomegalovirus and matched Connexin 26 peers

Objective

To compare the long-term speech perception and production outcomes after cochlear implantation (CI) in children deafened by congenital cytomegalovirus (cCMV) with a matched group of Cx26-CI children by controlling for chronological age and magnetic resonance imaging (MRI) findings.

Methods

Retrospective review of 12 cCMV-CI children and matched Cx26-CI children for speech perception and speech production outcomes.

Results

Two trends were seen in our data. First, cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years. The majority of cCMV-CI children with normal MRI scans (5 out of 7), suffered from a delayed-onset SNHL. Their mean age at first implantation (2y9m, range 15–82m) was higher compared to their matched Cx26 peers (9m, range 7–12m). Before being implanted, the majority of these delayed-onset hearing impaired children had benefited from a certain period of normal hearing (with or without amplification of a hearing aid). Possibly, this input might have led to an advantage the first three years after CI. Second, results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormalities on MRI scans catch up for speech perception, but lag behind for speech production.

Conclusion

cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years, whereas results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormal MRI scans catch up for speech perception, but lag behind for speech production. In future, the inclusion of MRI results may assist in improved counseling of parents with cCMV deafened children seeking CI.     

Diagnostic utility of magnetic resonance imaging and magnetic resonance angiography in the radiological evaluation of pulsatile tinnitus

Aim

Our aim was to assess the diagnostic utility of magnetic resonance imaging with complimentary magnetic resonance angiography (MRI/MRA) in the radiological evaluation of patients with pulsatile tinnitus (PT).

Materials and Methods

The present study was retrospectively conducted on 27 patients with pulsatile tinnitus. All patients showed normal otoscopic findings and were evaluated with magnetic resonance imaging (MRI) with complimentary magnetic resonance angiography (MRA), 9/27 (33.3%) patients were investigated by CT, and 12/27 (44.4%) were evaluated by angiography. All patients’ clinical investigation was reviewed to discard systemic causes of PT.

Results

All hard copies of MRI/MRA studies were evaluated. MRI/MRA detected the underlying etiology of subjective pulsatile tinnitus (PT) in 11/27 patients (40.7%), and 16/27 patients (59.5%) showed normal MRI/MRA examination. The most common cause was dural arteriovenous malformation (AFM) in 4/27 (14.8%) patients, high jugular bulbus in 2/27 (7.4%), aneurysm of internal carotid artery in 1/27 (3.7%), aberrant internal carotid artery in 1/27 (3.7%), vertebral artery hypoplasia in 2/27 (7.4%), and glomus tumor in 1/27 (3.7%). The statistical results of the present study showed that MRI/MRA had the following: 80% sensitivity, 88% specificity, 86% accuracy, 85% PPV, 83% NPV, and 15% error percentage for diagnosis of PT.

Conclusion

MRI/MRA was an effective radiological imaging method in detecting the underlying pathology of pulsatile tinnitus. Magnetic resonance may be considered a first line diagnostic imaging modality in the assessment of subjective pulsatile tinnitus.