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Current guidelines for patients with chronic lymphocytic leukemia (CLL) recommend mutation status determination of the clonotypic IGHVgene before treatment initiation to guide the choice of first-line therapy. Currently, commercially available next-generation sequencing (NGS) solutions have technical constraints, as they necessitate at least a 2 × 300 bp sequencing, which restricts their use for routine practice. The cost of the commercial kits also represents an important drawback. We present a new method called Next-CLL, a ready-to-use strategy to evaluate IGHVgene mutation status using any NGS device (including 2 × 150 bp sequencers) in routine diagnostic laboratories. The performance of Next-CLL was validated on genomic DNA and cDNA obtained from 80 patients with CLL at diagnosis. Next-CLL identified a productive clone in 100% of cases, whereas PCR with Sanger sequencing led to a 12.5% failure rate. Next-CLL had 100% concordance with the reference technique for IGHVgene identification and allowed assessment of the IGHVmutation status from the leader sequence, following international guidelines. Comparing a large retrospective series of samples, analyzed by using Sanger sequencing (n = 773) or Next-CLL (n= 352), showed no bias in IGHVusage or mutational status, further validating our strategy in the real-life setting. Next-CLL represents a straightforward workflow for IGHVanalysis in routine practice to assess clonal architecture and prognosis of patients with CLL.

Objectif: Description du deuxième cas de surdité brusque bilatérale avec fibrose intra-cochléaire objectivée compliquant une leucémie myéloïde chronique.

IMPORTANCE: The association between hearing impairment and cognitive decline has been established; however, the effect of cochlear implantation on cognition in profoundly deaf elderly patients is not known. OBJECTIVE: To analyze the relationship between cognitive function and hearing restoration with a cochlear implant in elderly patients. DESIGN, SETTING, AND PARTICIPANTS: Prospective longitudinal study performed in 10 tertiary referral centers between September 1, 2006, and June 30, 2009. The participants included 94 patients aged 65 to 85 years with profound, postlingual hearing loss who were evaluated before, 6 months after, and 12 months after cochlear implantation. INTERVENTIONS: Cochlear implantation and aural rehabilitation program. MAIN OUTCOMES AND MEASURES: Speech perception was measured using disyllabic word recognition tests in quiet and in noise settings. Cognitive function was assessed using a battery of 6 tests evaluating attention, memory, orientation, executive function, mental flexibility, and fluency (Mini-Mental State Examination, 5-word test, clock-drawing test, verbal fluency test, d2 test of attention, and Trail Making test parts A and B). Quality of life and depression were evaluated using the Nijmegen Cochlear Implant Questionnaire and the Geriatric Depression Scale-4. RESULTS: Cochlear implantation led to improvements in speech perception in quiet and in noise (at 6 months: in quiet, 42% score increase [95% CI, 35%-49%; P < .001]; in noise, at signal to noise ratio [SNR] +15 dB, 44% [95% CI, 36%-52%, P < .001], at SNR +10 dB, 37% [95% CI 30%-44%; P < .001], and at SNR +5 dB, 27% [95% CI, 20%-33%; P < .001]), quality of life, and Geriatric Depression Scale-4 scores (76% of patients gave responses indicating no depression at 12 months after implantation vs 59% before implantation; P = .02). Before cochlear implantation, 44% of the patients (40 of 91) had abnormal scores on 2 or 3 of 6 cognition tests. One year after implant, 81% of the subgroup (30 of 37) showed improved global cognitive function (no or 1 abnormal test score). Improved mean scores in all cognitive domains were observed as early as 6 months after cochlear implantation. Cognitive performance remained stable in the remaining 19% of the participants (7 of 37). Among patients with the best cognitive performance before implantation (ie, no or 1 abnormal cognitive test score), 24% (12 of 50) displayed a slight decline in cognitive performance. Multivariate analysis to examine the association between cognitive abilities before implantation and the variability in cochlear implant outcomes demonstrated a significant effect only between long-term memory and speech perception in noise at 12 months (SNR +15 dB, P = .01; SNR +10 dB, P < .001; and SNR +5 dB, P = .02). CONCLUSIONS AND RELEVANCE: Rehabilitation of hearing communication through cochlear implantation in elderly patients results in improvements in speech perception and cognitive abilities and positively influences their social activity and quality of life. Further research is needed to assess the long-term effect of cochlear implantation on cognitive decline.

To review indications, anatomical results, and complications of cochlear implant (CI) surgery in adults for which middle ear and mastoid obliterations were performed.

Objectives:We describe and evaluate the process of fixation of the Digisonic SP cochlear implant with two titanium screws.Methods:The characteristics of this implant allow cochlear implantation using a minimal incision, a subperiosteal pocket, and fixation with two titanium screws, without drilling a custom-fitted seat or creating suture-retaining holes in the skull. The fixation system relies on two tailfins for use of osseo-integratable screws, incorporated into the cochlear implant housing. The first version of this fixation system was modified after a case of device migration: The size of the titanium insert inside the silicone tailfin was increased. Data on 156 patients (8 months to 86 years of age) from a 4-year period in 6 cochlear implantation centers were retrospectively evaluated. Ten patients have undergone bilateral implantation.Results:Of 166 implantations, 4 postoperative infections and 1 device failure after head trauma were reported. No cerebrospinal fluid leaks or epidural hematomas were reported. One device migration was observed in the first series; no device migrations occurred in the second series.Conclusions:The fixation system with screws embedded in the Digisonic SP involves a fast and simple surgical technique that seems to efficiently prevent implant migration.

To assess audiological performance, satisfaction rate, and side effects of 100 patients who have been using the middle ear implant Vibrant Soundbridge (VSB) for 5 to 8 yr when compared with data collected from 3 to 18 mo postsurgery.

Les modalités de la réhabilitation auditive dépendent de la cause et de la sévérité de la surdité. Les surdités rattachées à des atteintes de l’oreille moyenne peuvent bénéficier de traitements médicaux ou chirurgicaux, par des prothèses ossiculaires, s’il est nécessaire de restaurer la fonction de la chaîne des osselets. Dans les surdités neurosensorielles, surdités d’oreille interne, l’utilisation d’aides auditives est d’emblée envisagée. Dans les cas pour lesquels elles sont insuffisantes, du fait de la sévérité de la surdité, ou non utilisables, pour des raisons d’intolérance, il est possible de recourir à des prothèses implantées ou à des implants cochléaires. Les indications des implants du tronc cérébral restent à ce jour limitées aux surdités totales bilatérales liées à une destruction complète des cochlées et des nerfs cochléaires. Ces orientations thérapeutiques sont choisies après une évaluation multidisciplinaire du malentendant, qui permet de caractériser l’atteinte auditive et son retentissement. Dans tous les cas, la restauration d’une audition bilatérale est à privilégier, dans le but d’améliorer la compréhension globale, en particulier dans les situations bruyantes, et de localiser les sources sonores.

To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults.

To evaluate the results of partial and total ossicular reconstruction prostheses entirely made of hydroxylapatite.

The purpose of this study was to analyze the imaging characteristics of 12 cholesterol granulomas as a function of their clinical symptoms. The results of the different surgical approaches and the management of these lesions are discussed.

Hearing loss, vertigo, and tinnitus have been related to arterial hypertension. The aim of the present work was to study the permeability of the blood-perilymph and of the labyrinthine barrier, between endolymph and perilymph, to small molecules during chronic and acute hypertension. Experiments were performed in normotensive Wistar-Kyoto (WKY) and spontaneously hypertensive rats (SHR). Acute hypertension was induced by phenylephrine. Perilymph was sampled from the first turn of the scala vestibuli and the Na, K, urea, and radioactive concentrations (14C-urea and 3H-mannitol) were measured. In another experimental set, the endocochlear potential was recorded from the basal turn of scala media, before and after phenylephrine injection. The composition of the perilymph and the kinetic constants for 14C-urea and 3H-mannitol were similar in WKY and SHR, and not modified after acute hypertension. In endolymph, the endocochlear potential in SHR (+80±2.7 mV, n=24) was lower (P<0.001) than in WKY (+98±1.5 mV, n=29). The endocochlear potential was decreased by 40 mV during acute hypertensive peak in seven out of 19 WKY but not in SHR rats (n=13). In conclusion, chronic or acute hypertension did not severely alter the permeability to small molecules of the blood-perilymph barrier. The relationship between the low endocochlear potential and hypertension in SHR remains to be evaluated. After acute hypertensive peak, the presence of vascular protective mechanisms in the cochlea could account for the stable endocochlear potential recorded in SHR and 60% of normotensive rats.