Ultrarare heterozygous pathogenic variants of genes causing dominant forms of early-onset deafness underlie severe presbycusis.

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

Transtympanic pseudoaneurysm of the internal carotid artery complicating a myringotomy in a four-year old child: Case report and literature review.

We report the first case of a transtympanic iatrogenic internal carotid artery (ICA) pseudoaneurysm diagnosed in a 4-year-old child following a myringotomy. An endovascular treatment with a covered-stent was decided; spontaneous thrombosis was found during the therapeutic arteriography, and the procedure was aborted. Otoscopy and computed tomography (CT) scan monitoring showed a prolonged thrombosis and the disappearance of the pseudoaneurysm 18months after the diagnostic arteriography. Based on literature review, endovascular techniques seem to be preferred to the surgical approach for treatment of intrapetrous ICA pseudoaneurysm, however clinical and CT scan monitoring may also be a valid option.

Long-term results of middle fossa plugging of superior semicircular canal dehiscences: clinically and instrumentally demonstrated efficiency in a retrospective series of 16 ears.

The objective of this study is to report the surgical outcome after middle fossa approach (MFA) plugging in patients suffering from a superior semi-circular canal dehiscence (SCD) syndrome. This is a retrospective case review. Tertiary referral center. Sixteen ears in 13 patients with a SCD syndrome suffering from severe and disabling vestibular symptoms with a bony dehiscence on CT scan >3 mm and decreased threshold of cervical vestibular evoked potentials (cVEMPs). We assessed preoperatively: clinical symptoms, hearing, cVEMPs threshold, size of dehiscence and videonystagmography (VNG) with caloric and 100 Hz vibratory tests. Postoperatively, we noted occurrences of neurosurgical complication, evolution of audiological and vestibular symptoms, and evaluation of cVEMP data. Tullio's phenomenon was observed in 13 cases (81.3 %) and subjectively reported hearing loss in seven (43.7 %). All patients were so disabled that they had to stop working. No neurosurgical complications were observed in the postoperative course. In three cases (16.6 %), an ipsilateral and transitory immediate postoperative vestibular deficit associated with a sensorineural hearing loss (SNHL) was noted, which totally resolved with steroids and bed rest. All patients were relieved of audiological and vestibular symptoms and could return to normal activity with a mean follow-up of 31.1 months (range 3-95). No patient had residual SNHL. cVEMPs were performed in 14 ears postoperatively and were normalized in 12 (85.7 %). Two of the three patients operated on both sides kept some degree of unsteadiness and oscillopsia. MFA plugging of the superior semi-circular canal is an efficient and non-hearing deteriorating procedure.

Effect of corticosteroids on facial function after cerebellopontine angle tumor removal: a double-blind study versus placebo.

The aim of this study was to assess the effect of corticosteroids administered intra- and postoperatively on the occurrence of facial palsy after a cerebellopontine angle (CPA) tumor resection, and to investigate pre- and intraoperative prognostic factors. A multicenter, prospective, randomized, double-blind and versus-placebo study was conducted between 2006 and 2010. Three hundred and ten patients operated on for a CPA tumor (96% vestibular schwannomas, 4% miscellaneous) were included by five participating centers. The population was stratified into patients with small (≤15 mm CPA on axial MRI views) and large tumors. In each group, patients were randomized to receive corticosteroid (1 mg/kg/day i.v. methylprednisolone intraoperatively and at postoperative days 1-5) or placebo. Steroids did not affect the facial function at postoperative days 1, 8 and 30 in patients with small or large tumors as evaluated by House and Brackmann grading.

Prevalence of vestibular disease in France: analysis of prescription data from a national health insurance database.

Vestibular problems are frequent reasons for primary care consultations. However, there is considerable uncertainty about the prevalence and cost of vestibular disorders. Despite ambiguous effectiveness data, the histamine analogue betahistine is widely and almost exclusively used for treatment of vertigo. Prescription of betahistine can, therefore, be used as a proxy estimate for prevalence. We used openly available claims data from the French health insurance data warehouse, defining annual prevalence of vestibular disease as the number of people who received at least one betahistine prescription that year. Dosage and pack size of each prescribed formulation were extracted to calculate the sum of betahistine in mg and the Defined Daily Dose (DDD) for age and sex strata and in total. To estimate the relative impact of one landmark trial, the BEMED study, we compared prescriptions from the years 2014/2015 to prescriptions in 2019/2022. A total of 735,121 (2014), 694,705 (2015), 614,431 (2019), and 562,476 (2022) persons filled in a prescription of betahistine. Patients were predominantly older and female. Average amount dispensed per year and per person increased from 4422.54 mg during the pre-BEMED period to 4736.90 mg during the post-BEMED period. DDD decreased from 130 Mio per year in 2014/2015 to 116 Mio per year in 2019/2022. Total costs for betahistine decreased by 42% from 21,615,037 Euro in 2014 to 12,894,249 Euro in 2022. Vestibular disease is frequent in France and has a relevant impact on population health. Despite conflicting clinical evidence, betahistine continues to be prescribed widely in medical practice.

Guidelines of the French Society of Otorhinolaryngology and Head and Neck Surgery (SFORL) for vestibular rehabilitation in children with vestibular dysfunction. A systematic review.

INTRODUCTION: The consequence of complete or partial uncompensated vestibular dysfunction in children is usually balance disorders, with the risk of falls and increased fatigue, particularly during tasks requiring postural control. The aim of these recommendations is to establish guidelines for vestibular rehabilitation (VR) in children with vestibular impairment. MATERIAL AND METHODS: The guidelines were developed based on a systematic review of the international literature, validated by a multidisciplinary group of French-speaking otorhinolaryngologists, scientists, and physiotherapists. They are classified as grade A, B, C, or expert opinion according to a decreasing level of scientific evidence. RESULTS: A PubMed search of studies published between January 1990 and December 2021 was carried out using the keywords "vestibular," "rehabilitation," and "children". After filtering and reviewing the articles, a total of 10 publications were included to establish the recommendations. CONCLUSION: It is recommended that a vestibular assessment be carried out before VR, including a study of vestibulo-ocular reflex, otolithic function, and postural control. In cases of vestibular dysfunction, physiotherapy treatment is recommended from an early age to train different aspects of postural control, including anticipatory and reactive postural adjustments. VR adapted to the pediatric population is recommended for children whose vestibular dysfunction leads to functional disorders or symptoms of vertigo for those who have suffered head trauma. It is recommended that children with bilateral vestibular impairment be treated using gaze stabilization exercises for adaptation and substitution. Optokinetic stimulation and virtual reality are not recommended for children and young adolescents.

Comparison between bilateral cochlear implants and Neurelec Digisonic(®) SP Binaural cochlear implant: speech perception, sound localization and patient self-assessment.

An alternative to bilateral cochlear implantation is offered by the Neurelec Digisonic(®) SP Binaural cochlear implant, which allows stimulation of both cochleae within a single device. The purpose of this prospective study was to compare a group of Neurelec Digisonic(®) SP Binaural implant users (denoted BINAURAL group, n = 7) with a group of bilateral adult cochlear implant users (denoted BILATERAL group, n = 6) in terms of speech perception, sound localization, and self-assessment of health status and hearing disability. Speech perception was assessed using word recognition at 60 dB SPL in quiet and in a 'cocktail party' noise delivered through five loudspeakers in the hemi-sound field facing the patient (signal-to-noise ratio = +10 dB). The sound localization task was to determine the source of a sound stimulus among five speakers positioned between -90° and +90° from midline. Change in health status was assessed using the Glasgow Benefit Inventory and hearing disability was evaluated with the Abbreviated Profile of Hearing Aid Benefit. Speech perception was not statistically different between the two groups, even though there was a trend in favor of the BINAURAL group (mean percent word recognition in the BINAURAL and BILATERAL groups: 70 vs. 56.7% in quiet, 55.7 vs. 43.3% in noise). There was also no significant difference with regard to performance in sound localization and self-assessment of health status and hearing disability. On the basis of the BINAURAL group's performance in hearing tasks involving the detection of interaural differences, implantation with the Neurelec Digisonic(®) SP Binaural implant may be considered to restore effective binaural hearing. Based on these first comparative results, this device seems to provide benefits similar to those of traditional bilateral cochlear implantation, with a new approach to stimulate both auditory nerves.

Lipochoristomas of the cerebellopontine angle and internal acoustic meatus: a seven-case review.

BACKGROUND: Intracranial lipomas are extremely rare tumors, with certain clinical and radiological characteristics. METHODS: We considered the diagnostic evaluation and treatment options of seven patients presenting with lipoma in the cerebellopontine angle or internal acoustic meatus in our department. RESULTS: Mean age was 51, with four out of seven cases being women. Balance disorders (vertigo, dizziness) were the predominant symptoms (in six out of seven patients), followed by hearing loss. The diagnosis of intracranial lipomas was based on the results of imaging studies, especially on magnetic resonance imaging. The management of lipomas of the CPA and of the IAM should initially be conservative, including close follow-up of the patient and evaluation of a potential change in tumor size. Surgical management of CPA lipomas should be reserved for patients with intractable clinical symptoms due to tumor overgrowth. CONCLUSIONS: Diagnosis of CPA and IAM lipomas is considered to be a clinical and imaging challenge. As the complete resection of such lipomas is a risky choice and taking into account the benign course of such tumors, the only absolute surgical indication should be uncontrolled tumor growth.

Endolymphatic sac tumors: experience of three cases.

OBJECTIVES: To describe the clinical features, radiological findings, treatment and outcomes of three cases of endolymphatic sac tumors (ELST). METHODS: Retrospective analysis of three cases of ELST. RESULTS: The first patient had a large ELST invading the labyrinth after a long history of vertigo. He was recurrence-free 1 year after retrolabyrinthine surgical removal. In the second case, an acute peripheral facial nerve paralysis associated with ipsilateral sensorineural hearing loss led to the diagnosis. A translabyrinthine approach was used to remove the tumor, which recurred three times over 10 years. The third patient was a young woman suffering from von Hippel-Lindau (VHL) disease and referred for a sudden sensorineural hearing loss due to an intralabyrinthine hemorrhage secondary to a 2 mm-large endolymphatic sac-confined ELST. Her hearing was totally lost after the deafness recurred 1 month after this first episode. MRI demonstrated a small bilateral ELST. The patient refused surgery on the deaf side. CONCLUSION: ELST are difficult to diagnose due to the wide variety of their presentations. Patients with ELST should be screened for VHL disease. Dural invasion and tumor hypervascularization increase the risk of local recurrences after surgery. Early surgical resection may lead to complete tumor removal and inner ear preservation.

Anatomo-radiological study of the superior semicircular canal dehiscence of 37 cadaver temporal bones.

PURPOSE: To assess the presence of dehiscence of the superior semicircular canal (SSCC) on computed tomography (CT) scanning and to study the microscopic anatomo-radiological correlation. MATERIALS AND METHODS: Thirty-seven temporal bones preserved in formalin, regardless of the clinical history of cadavers, were studied. A microscopic anatomical study was conducted with an operative microscope (20×). The settings of the CT permitted to obtain 0.6 mm slices contiguous reconstruction in Pöschl plane and in Stenvers plane. Three-dimensional (3D) reconstructions were performed if a radiological dehiscence was observed. The apex thickness was measured in Pöschl plane. The radiological positive criterion of SSCC dehiscence was an absence of bone coverage of more than 1 mm long in Pöschl and Stenvers planes. RESULTS: We observed three dehiscences of the 37 temporal bones on CT in Pöschl and Stenvers planes. However, no dehiscence was found microscopically. The 3D reconstruction was also positive in these three cases. Reconstructions in the Pöschl plane offered good results up to a bone thickness of 0.6 mm. When it was lower than 0.6 mm, the interpretation of the images appeared to be subjective. CONCLUSION: This study emphasizes the limitations of CT imaging, with a risk of false positives to take into account when interpreting the images. The 3D reconstructions also give too many false positives to be used alone and make an accurate diagnosis. The diagnosis of SSCC dehiscence will therefore remain clinical. Complementary and instrumental radiological examinations should be performed only to confirm this clinical suspicion.

Unique case of malignant transformation of a vestibular schwannoma after fractionated radiotherapy.

OBJECTIVE: Malignant transformation of vestibular schwannoma is considered a rare clinical entity. Radiotherapy, as a treatment option for vestibular schwannoma, is regarded as a potential risk factor for secondary malignancy. Recently, radiotherapy with dose fractionation has been proposed, intended to diminish the risk of radiation-induced neuropathy. CASE PRESENTATION: The aim of the present study is to report the first case, to the best of our knowledge, of malignant transformation of a residual vestibular schwannoma 19 years after fractionated radiotherapy, describing its characteristics with regard to those previously reported in the literature. CONCLUSIONS: The main purpose of the present work is to state that the knowledge of the iatrogenic potential pitfalls of any technique of radiotherapy in clinical oncology is becoming a necessity. Finally, our report demonstrates that the irradiated patients must be monitored for life because a secondary malignancy may appear after a very long delay.

Spontaneous osteodural defects of the temporal bone: diagnosis and management of 12 cases.

OBJECTIVES: The objectives of the study were to describe the clinical presentation, diagnostic process, surgical treatment, and outcome of patients with spontaneous dural defect of temporal bone and to explore the possible contribution of idiopathic intracranial hypertension. METHODS: Medical records of consecutive patients with spontaneous defects of the temporal bone were reviewed. Clinical presentation, diagnostic process, exploration of benign intracranial hypertension, surgical management, and outcome of patients are presented. RESULTS: Six of the 12 patients presenting with spontaneous cerebrospinal fluid otorrhea were women. Ages ranged from 38 to 76 years. Seven patients presented with meningitis. The location and the extent of the occurring defect were detected by computed tomography in all cases. Radiologic signs of empty sella syndrome, indicator of benign intracranial hypertension, were revealed in 3 cases using magnetic resonance imaging. Six tegmen defects were repaired using a middle fossa approach without recurrence. Four patients received the combined approach. All patients had complete resolution of the cerebrospinal fluid leak, although 2 cases developed adverse effects attributable to surgical procedure. CONCLUSION: The diagnosis of spontaneous cerebrospinal fluid otorrhea requires clinical suspicion in the setting of meningitis and persistent serous otitis media. High-resolution computed tomography can confirm the diagnosis. The authors' findings advocate the multilayered closure technique through a middle fossa approach.

Positive Impact of Genetic Test on the Management and Outcome of Patients With Paraganglioma and/or Pheochromocytoma.

CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are characterized by a strong genetic component, with up to 40% of patients carrying a germline mutation in a PPGL susceptibility gene. International guidelines recommend that genetic screening be proposed to all patients with PPGL. OBJECTIVE: Our objective was to evaluate how a positive genetic test impacts the management and outcome of patients with SDHx or VHL-related PPGL. DESIGN: We performed a multicentric retrospective study involving 221 propositi carrying an SDHB, SDHD, SDHC, or VHL germline mutation. Patients were divided into two groups: genetic patients, who were informed of their genetic status within the year following the first PPGL diagnosis, and historic patients, who only benefited from the genetic test several years after initial PPGL diagnosis. RESULTS: Genetic patients had better follow-up than historic patients, with a greater number of examinations and a reduced number of patients lost to follow-up (9.6% vs 72%, respectively). During follow-up, smaller (18.7 vs 27.6 mm; P = 0.0128) new PPGLs and metastases as well as lower metastatic spread were observed in genetic patients. Of note, these differences were reversed in the historic cohort after genetic testing. Genetic patients who developed metachronous metastases had a better 5-year survival rate than historic patients (P = 0.0127). CONCLUSION: Altogether, our data suggest that early knowledge of genetic status had a positive impact on the management and clinical outcome of patients with a germline SDHx or VHL mutation.

Zinc protection against pneumolysin toxicity on rat cochlear hair cells.

Streptococcus pneumoniae can induce local and systemic diseases such as meningitis, otitis media, and pneumonia. One third of these meningitis cases can be associated with irreversible sensorineural hearing loss whose mechanisms likely involves the exotoxin pneumolysin (PLY) that irreversibly damages cochlear hair cells (HCs). In the respiratory system and in neuron it has been demonstrated that zinc deficiency increases severity and mortality of such infections in animal models and in children. Moreover, zinc supplementation can decrease the severity of pneumococcal respiratory infections. The aim of our study was to assess the potential protective effect of zinc against PLY toxicity on HCs in culture. Our results showed that in the presence of zinc at concentration as low as 1 microM, the toxicity of PLY was largely reduced by about 50% for both inner and outer HCs. At 300 microM of zinc, protection significantly increased with 62 and 55.2% for IHCs and OHCs, respectively. Our results suggest that the protective effect of zinc is likely due to an inhibition of the toxin incorporation and aggregation into the plasma membrane, thus preventing calcium influx through the toxin pores. Our findings raise the possibility that treatments with zinc may help to prevent debilitating otological sequelae from pneumococcal infection.

Middle Ear Osteoma Causing Mixed Hearing Loss: A Case Report.

Osteomas of the middle ear are rare benign tumors. Their consequences and symptoms are due to their specific location, such as the promontory or the epitympanum and their contact with the facial nerve, the semicircular canal, the ossicles, and the oval or round windows. We report a very unusual case of middle ear osteoma (MEO) in a 23-year-old male patient causing a right mixed hearing loss by contacting and overwhelming the incus and stapes. The lesion was also closely attached to the tympanic portion of the fallopian canal. Since the stapes was not clearly visible behind the lesion, careful observation was preferred to surgery owing to the high risk of inner ear damage and facial palsy with removal of the lesion. MEOs are rarely situated at this critical site. Regular clinical and computerized tomography monitoring is warranted to check their growth. This case also supports the etiological theory of chronic middle ear inflammation causing osteomas.

Multifrequency immittancemetry in experimentally induced stapes, round window and cochlear lesions.

OBJECTIVES: To establish that admittance (Y) and susceptance (B) conductance (G) tympanograms at 2 kHz can reflect the status of the annular ligament and the cochlear pressure. METHODS: Seven experiments were set up in 22 guinea pigs: ventilation of the bulla, blockage of the stapes and round window membrane (RWM), fistula, fluid removal from the cochlea, injection of saline in the scala tympani and acoustic trauma. Resonance frequency, Y, B and G at 2 kHz and curve shapes were analyzed before and after lesions. RESULTS: A supplementary peak was observed in Y/G tympanograms in all RWM fistulas and in some cases of acoustic trauma; injection of saline into the scala tympani induced constant, immediate and reproducible changes; RWM and stapes blockages induced foreseeable peaking at 2 kHz; fluid removal from the cochlea induced multiple peak curves. CONCLUSION: Experimentally induced modifications at the AL result in noticeable, constant and reproducible changes in tympanogram curves at 2 kHz and seem to reflect inner ear pressure.

Transpetrosal approaches for meningiomas of the posterior aspect of the petrous bone Results in 43 consecutive patients.

OBJECTIVE: To assess outcome following excision of meningiomas of the posterior aspect of the petrous bone through transpetrosal approaches. MATERIAL AND METHOD: We carried out a retrospective case-series study in a multidisciplinary tertiary care center on all patients who underwent meningiomas removal from January 1989 to September 2005. Surgical approaches were transpetrosal: widened retrolabyrinthine, translabyrinthine, transotic and transcochlear, occasionally combined with a subtemporal transtentorial approach. Epidemiology, symptoms, preoperative evaluation, surgery, postoperative complications and facial and auditory results were analyzed using standardized grading systems. The Desgeorges and Sterkers classification was used to assess tumor size and location. RESULTS: Forty women and three men underwent surgery (mean age: 56.7). Medium-sized tumors stages 2 and 3 (84%) and AM and P localization (34% and 20.4%) predominated. In 65% of cases, the tumor extended beyond the CPA. Main presenting symptoms were balance disorders (72%) and sensorineural hearing loss (53.5%). Mortality was nil. A preoperative facial nerve paresis was present in 14% of patients. Tumor removal was complete in 79.1% of cases. At 1-year post-op, 73% of patients had a normal or subnormal facial function and 55% had serviceable hearing. A cerebrospinal fluid leakage occurred in 6.9%. DISCUSSION: Posteriorly attached meningiomas are less symptomatic and of better prognosis than medially inserted ones. Transpetrosal approaches are reliable for the removal for all types and sizes of such tumors, and can be easily combined in the same procedure with a subtemporal transtentorial approach to remove extensions to the clivus and tentorium. They offer low morbidity and a high proportion of facial nerve and hearing preservation.

Intracochlear hemorrhage after gamma knife radiosurgery.

PURPOSE: To describe an acute complication after gamma knife stereotactic radiosurgery (GKRS) for vestibular schwannoma (VS) in a neurofibromatosis type 2 (NF2) patient. STUDY DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 20-year-old man, who had bilateral VS and was having right-sided profound deafness, underwent GKRS for a 2-cm left-sided VS in an attempt to preserve his only hearing ear. He received a margin dose of 13 Gy to the 50% isodose line. Twenty-four hours after treatment, he presented with spinning vertigo, left-sided dead ear, and ipsilateral mild facial paralysis (House-Brackmann grade 3). RESULTS: Magnetic resonance imaging demonstrated an intracochlear hemorrhage at the level of the basal turn of the left cochlea. Hearing did not recover, and the patient had to resort to lip reading. The facial paralysis regressed completely after 3 months. CONCLUSION: Many cases of hemorrhage caused by GKRS have previously been reported, but all were related to meningiomas or brain metastases. Because the patient had no coagulation defect, sign of trauma, or any history of infection, the hemorrhage might have been caused by a direct thermal effect on the endothelial cells or to an immediate tumoral swelling, inducing an increase in intravascular outflow resistance and leading to venous obliteration. To our knowledge, this is the first report of acute intracochlear hemorrhage after GKRS for VS.

Necrotizing external otitis: a report of 46 cases.

OBJECTIVES: To define germs involved, clinical presentation, treatment regimen, and prognostic factors in necrotizing external otitis. PATIENTS AND METHODS: Retrospective study reviewing a series of 46 patients treated during 10 years in a tertiary care center. Diagnosis was confirmed by using otomicroscopy, computed tomographic and/or magnetic resonance imaging scan and bone scintigraphy (Te 99 and Ga 26 bone scan). Patients were provided ceftazidime and ciprofloxacin intravenously and monitored using a Ga-67 bone scan. The following were assessed: presenting symptoms, general context, bacteriological analysis, imaging protocol sensitivity, complications, delay to healing, and cure rate. RESULTS: Sex ratio was 2.29 (mean age, 73.6 yr). The most common presenting symptoms were otalgia and otorrhea in 97.8 and 91.3%, respectively. Facial paralysis was present in 19.6% of cases. Thirty patients had diabetes mellitus (65.2%), and 8 were immunocompromised (17.4%). Pseudomonas aeruginosa was isolated in 69.2% of cases and was resistant to ciprofloxacin in 18.5%, but was susceptible to ceftazidime in all cases. Four patients died during the treatment (4.4%), but only 2 of disease. The mean delay to healing was 14 weeks (SD, 9.7). Healing rate was 95.6%. No relapse was observed after a mean follow-up of 78.4 weeks (SD, 36.5 wks). Two factors significantly influenced the prognosis: facial paralysis and existence of systemic factors for immune deficiency (p = 0.023 and 0.038, respectively). CONCLUSION: The association of ciprofloxacin and ceftazidime was efficient in countering the increasing resistance of P. aeruginosa to quinolones. We propose a prognostic classification of necrotizing external otitis based on the presence of facial paralysis and/or systemic factors.