Three Years of Vestibular Infant Screening in Infants With Sensorineural Hearing Loss.

OBJECTIVES: Although vestibular deficits are more prevalent in hearing-impaired children and can affect their development on many levels, a pediatric vestibular assessment is still uncommon in clinical practice. Since early detection may allow for timely intervention, this pioneer project has implemented a basic vestibular screening test for each six-month-old hearing-impaired infant in Flanders, Belgium. This study aims to report the vestibular screening results over a period of three years and to define the most important risk factors for abnormal vestibular screening results. METHODS: Cervical Vestibular Evoked Myogenic Potentials with bone-conduction were used as a vestibular screening tool in all reference centers affiliated to the Universal Newborn Hearing Screening Program in Flanders. From June 2018 until June 2021, 254 infants (mean age: 7.4 months, standard deviation: 2.4 months) with sensorineural hearing loss were included. RESULTS: Overall, abnormal vestibular screening results were found in 13.8% (35 of 254) of the infants. The most important group at risk for abnormal vestibular screening results were infants with unilateral or bilateral severe to profound sensorineural hearing loss (20.8%, 32 of 154) (P < .001, odds ratio = 9.16). Moreover, abnormal vestibular screening results were more prevalent in infants with hearing loss caused by meningitis (66.7%, 2 of 3), syndromes (28.6%, 8 of 28), congenital cytomegalovirus infection (20.0%, 8 of 40), and cochleovestibular anomalies (19.2%, 5 of 26). CONCLUSIONS: The vestibular screening results in infants with sensorineural hearing loss indicate the highest risk for vestibular deficits in severe to profound hearing loss, and certain underlying etiologies of hearing loss, such as meningitis, syndromes, congenital cytomegalovirus, and cochleovestibular anomalies.

Total ossicular chain reconstruction using a titanium prosthesis: functional results.

PURPOSE: To evaluate the efficacy and safety of a titanium total prosthesis (TORP) in ossicular chain reconstruction. METHODS: Retrospective analysis of 113 cases of total ossicular chain reconstruction performed by a single surgeon between 2006 and 2018. Follow-up lasted until January 2020. Participants were patients with chronic otitis media, cholesteatoma, or ossicular chain disruption. RESULTS: Mean preoperative air-bone gap (± standard deviation) was 32.74 dB (± 11.62). Mean postoperative air-bone gap was 21.68 (± 11.29). Mean air-bone gap improvement was 11.06 dB (± 14.99) (p < 0.001). 57 cases had a post-operative air-bone gap smaller than or equal to 20 dB (50.44%). In four cases, prosthesis dislocation was observed (12.39%). Mean prosthesis length in this group was 5.29 mm (± 0.86) versus 4.36 mm (± 1.02) in cases without prosthesis dislocation (p = 0.002). CONCLUSIONS: Total ossicular reconstruction using a titanium prosthesis yields favorable functional results, also during prolonged follow-up. Prosthesis length was significantly longer in cases with prosthesis dislocation.

Children with posterior semicircular canal dehiscence: A case series.

Posterior semicircular canal dehiscence is a rare condition and can cause a variety of symptoms. We report three cases of children between 5 and 12 years of age with a PSCD. They all presented with different complaints as follows: Tullio phenomenon in the first case, conductive hearing loss in the second and, conductive hearing loss and pulsatile tinnitus in the third. Imaging showed in all cases a PSCD on the right side, caused by a prominent jugular bulb (high riding bulb). We describe the clinical, audiometric and radiological findings, and discuss the management and therapy. A conservative "wait and see" approach is recommended, especially with children, because of the possible complications of surgery and the possibility that the symptoms will lessen with the skull base osseous maturation.

Middle ear reconstruction using the titanium Kurz Variac partial ossicular replacement prosthesis: functional results.

IMPORTANCE: Satisfactory functional results following ossicular chain reconstruction mainly depend on a stable connection between the tympanic membrane and the stapes, which is in turn dependent on the type of prosthesis used. Knowledge about the safety and functional outcome of the commercially available middle ear prostheses is therefore of great importance. OBJECTIVE: To evaluate the efficacy and safety of the Kurz TTP-Variac System partial ossicular replacement prosthesis (PORP) in ossiculoplasty. DESIGN, SETTING, AND PARTICIPANTS: Retrospective review of all ossiculoplasties performed by 1 surgeon at a secondary referral center from August 2006 through July 2012. Participants were patients with cholesteatoma, chronic otitis media, or ossicular chain disruption in the absence of inflammatory disease who underwent ossicular reconstruction. EXPOSURE Ossiculoplasty using a Kurz TTP-Variac System PORP. MAIN OUTCOMES AND MEASURES: Mean preoperative and postoperative air-bone gaps (ABGs) and improvements in ABG were analyzed for each frequency by means of a 4-frequency pure-tone average. Successful postoperative hearing was defined as postoperative ABG smaller than 20 dB. RESULTS: Eighty-nine ears in 83 patients aged 7 to 85 years were included. Transmeatal tympanoplasty was performed in 17 ears (19%). Seven ears (8%) underwent tympanoplasty with canal wall-down mastoidectomy, and 65 ears (73%) underwent canal wall-up (combined approach) tympanoplasty with mastoidectomy. The study population comprised 61 primary tympanoplasties (69%) and 28 revision cases (31%). Mean follow-up was 13 months. Overall, the ABG significantly improved from a mean (SD; range) of 26.19 (11.53; 3.75-51.25) dB to 15.58 (9.80; 0-48.75) dB (P < .01 for all frequencies). Mean ABG improvement was 10.62 dB. Successful postoperative hearing was obtained in 65 ears (73%). Revision surgery, especially in ears with ossicular chain disruption without inflammatory disease, was associated with poorer functional outcome, whereas preservation of the malleus was associated with a better functional outcome (P < .05). There were few complications (1 prosthesis extrusion, 2 prosthesis dislocations, 2 reperforations, 3 cases of residual cholesteatoma, and 3 of light sensorineural hearing loss). CONCLUSIONS AND RELEVANCE: The titanium Kurz TTP-Variac System PORP is an effective prosthesis to reconstruct the ossicular chain. Complications are rare, illustrating the safety of the prosthesis.

Simultaneous true stapes fixation and bilateral bony dehiscence between the internal carotid artery and the apex of the cochlea: the ultimate pitfall.

BACKGROUND: A dehiscence of a semicircular canal can mimic the audiologic characteristics of otosclerosis. OBJECTIVE: To present a unique case report of a patient presenting with stapes fixation due to otosclerosis but persisting conductive hearing loss after uneventful laser-assisted stapedotomy with interposition of an àWengen titanium clip stapes prosthesis. Eventually, a bilateral bony dehiscence between the apical turn of the cochlea and the internal carotid artery was eventually identified, which explained the persisting postoperative conductive hearing loss. A dehiscence of the semicircular canals was duly ruled out preoperatively. PATIENT: : Caucasian man aged 63 years. INTERVENTION: Laser-assisted stapedotomy with interposition of an àWengen titanium clip stapes prosthesis. Later on, the patient received a Bone-Anchored Hearing Aid. RESULTS: A 63-year-old man presented with a clinical and audiologic bilateral stapes fixation due to otosclerosis. A possible dehiscence of a semicircular canal was duly ruled out preoperatively by high-resolution computed tomographic scanning. The stapes fixation was treated lege artis by laser-assisted stapedotomy and subsequent interposition of an àWengen titanium clip stapes prosthesis, but the mixed hearing loss persisted. Eventually, a dehiscence between the apical turn of the cochlea and the internal carotid artery was identified. Thereafter, the patient was adequately helped with a Bone-Anchored Hearing Aid. CONCLUSION: We think that this is the first published case of simultaneous occurrence of radiologically and preoperatively confirmed stapes fixation and bilateral bony dehiscence between the apex of the cochlea and the internal carotid artery. Preoperative imaging studies therefore should look not only for a possible dehiscence of a semicircular canal, mimicking otosclerosis, but also for a dehiscence between the cochlea and the internal carotid artery.

CO2 laser-assisted stapedotomy combined with àWengen titanium clip stapes prosthesis: superior short-term results.

OBJECTIVE: To report on the short-term results of CO2-laser assisted stapedotomy combined with the àWengen titanium clip stapes prosthesis. A comparison with published series using other prostheses and/or different stapedotomy techniques is made. STUDY DESIGN: Retrospective case series. PATIENTS: Patients with a history and audiologic data matching stapes fixation and computed tomographic imaging excluding other anomalies such as malleus fixation, dehiscent superior semicircular canal, and large vestibular aqueduct that may mimic stapes fixation-like hearing loss. INTERVENTION: All patients underwent CO2 laser-assisted stapedotomy (Lumenis Co. Israel CO2 laser, Acuspot 712, SurgiTouch 870 scanner) and subsequent reconstruction by means of the àWengen titanium clip stapes prosthesis by Heinz Kurz Medizintechnik GmbH (Germany). OUTCOME MEASURES: Comparison and statistical analysis of preoperative and postoperative audiologic data. RESULTS: Sixty-two stapedotomies were performed (61 patients) using the CO2 laser and àWengen titanium clip stapes prosthesis. The mean postoperative air-bone gap 3 months postoperatively was 5.1 +/- 0.5 dB (standard deviation [SD], 4.1 dB; 0.5, 1, 2, 4 kHz). Air-bone gap closure less than or equal to 10 dB was achieved in 54 cases (87%). Air-bone gap closure less than 20 dB was achieved in all cases. The average gain was 27.8 +/- 1.5 dB (SD, 12 dB; 0.5, 1, 2, 4 kHz). The average bone-conduction threshold shift or "overclosure" on 2,000 Hz was 13.6 +/- 1.3 dB (SD, 10 dB). There was no postoperative perceptive hearing loss exceeding 15 dB on any measured frequency. The Amsterdam Hearing Evaluation Plots have also been used to evaluate our data. These data were statistically analyzed and compare favorably to other published series. CONCLUSION: The authors conclude that the combination of CO2 laser-assisted stapedotomy and the àWengen titanium clip stapes prosthesis is a combination likely to yield superior results in experienced hands.

Primary angiosarcoma of the non-irradiated parotid gland: a most uncommon, highly malignant tumor.

This case report discusses the clinical presentation, imaging, surgery and further treatment and course of a primary angiosarcoma of a non-irradiated parotid gland.

Acoustic neuroma ingrowth in the cochlear nerve: does it influence the clinical presentation?

We examined the clinical presentation in patients with a histologically proven ingrowth of the cochlear nerve by acoustic neuroma to see whether this differs from what is known from large acoustic neuroma series. In total, 85 acoustic neuromas had an en bloc dissection to study histologically the relation between the cochlear nerve and the acoustic neuroma. In 21 of these 85 specimens, there was histologic proof of invasion of the cochlear nerve by the tumor. For 13 of these 21 tumors, sufficient clinical data could be retrieved to describe the clinical presentation in these patients. We collected clinical data such as age, sex, presenting symptoms, duration of symptoms, tone audiograms, tumor size measurements and volumetric calculations, and latency interval data I-V of brain stem evoked response audiometry and calculated whether there was any correlation among those data. We also compared these clinical data with the data from some large acoustic neuroma series. No clear difference could be shown between the clinical presentation of acoustic neuroma patients with cochlear nerve ingrowth and the clinical presentations in large acoustic neuroma series. This outcome favors the theory that the hearing impairment in acoustic neuroma patients is mainly the result of compression on the vessels of the cochlea and/or on the cochlear nerve.