Giant prolactinomas, a detailed analysis of 196 adult cases.

PURPOSE: Giant prolactinomas are a rare entity, representing approximately 5% of all prolactinomas. A systematic review of 196 adult cases was performed. A comparison of the clinical, biochemical and radiological characteristics, management and therapeutic outcomes in men versus women is made. METHODS: A structured search was conducted using the term 'giant prolactinoma'. Following inclusion criteria were used: diameter ≥ 40 mm, prolactin levels > 1000 ng/ml and no concomitant GH/ ACTH secretion. RESULTS: 196 cases were included [age: 38 (28-50) years, F/M ratio: 1/3.6]. Median tumor diameter was 53 (43-69) mm. Pituitary deficiency was present in 91% of cases, with hypogonadotropic hypogonadism being the most frequent. Most common presenting symptoms were visual impairment (73%) and headache (50%) in men and amenorrhea (58%) in women. 82% of cases were treated with a dopamine agonist (DA) as first-line treatment which led to normoprolactinemia, tumor shrinkage and visual improvement in 51%, 88% and 85% of cases, respectively. Surgery was performed in 29% of cases and all showed tumor remnant and persistent hyperprolactinemia. Women had a lower prolactin level and a smaller tumor diameter at diagnosis but pituitary deficiencies were more frequent and outcome was worse. CONCLUSION: Giant prolactinomas are rare and have a male predominance. Visual impairment is the most frequent presenting symptom in men and amenorrhea in women. The gender-related difference in tumor size and level of prolactin was confirmed in this analysis where men had a larger diameter and a higher baseline prolactin level. DAs are the treatment of choice, irrespective of tumor size and presence of visual impairment. As only half of the cases achieved normoprolactinemia we do not, in contrast to previous literature, state giant prolactinomas to be exquisitely sensitive to DAs. Patient characteristics associated with persistent hyperprolactinemia after treatment with a DA were female gender, higher baseline prolactin and larger tumor size . This analysis did show TSH- and ACTH-deficiency to be more frequent after surgery which was not seen for LH/FSH deficiency.

Evaluation of a Less Invasive Cochlear Implant Surgery in OPA1 Mutations Provoking Deafblindness.

Cochlear implantation (CI) for deafblindness may have more impact than for non-syndromic hearing loss. Deafblind patients have a double handicap in a society that is more and more empowered by fast communication. CI is a remedy for deafness, but requires revision surgery every 20 to 25 years, and thus placement should be minimally invasive. Furthermore, failed reimplantation surgery will have more impact on a deafblind person. In this context, we assessed the safety of minimally invasive robotically assisted cochlear implant surgery (RACIS) for the first time in a deafblind patient. Standard pure tone audiometry and speech audiometry were performed in a patient with deafblindness as part of this robotic-assisted CI study before and after surgery. This patient, with an optic atrophy 1 (OPA1) (OMIM#165500) mutation consented to RACIS for the second (contralateral) CI. The applicability and safety of RACIS were evaluated as well as her subjective opinion on her disability. RACIS was uneventful with successful surgical and auditory outcomes in this case of deafblindness due to the OPA1 mutation. RACIS appears to be a safe and beneficial intervention to increase communication skills in the cases of deafblindness due to an OPA1 mutation. The use of RACIS use should be widespread in deafblindness as it minimizes surgical trauma and possible failures.

Minimally Traumatic Cochlear Implant Surgery: Expert Opinion in 2010 and 2020.

This study aimed to discover expert opinion on the surgical techniques and materials most likely to achieve maximum postoperative residual hearing preservation in cochlear implant (CI) surgery and to determine how these opinions have changed since 2010. A previously published questionnaire used in a study published in 2010 was adapted and expanded. The questionnaire was distributed to an international group of experienced CI surgeons. Present results were compared, via descriptive statistics, to those from the 2010 survey. Eighteen surgeons completed the questionnaire. Respondents clearly favored the following: round window insertion, slow array insertion, and the peri- and postoperative use of systematic antibiotics. Insertion depth was regarded as important, and electrode arrays less likely to induce trauma were preferred. The usefulness of dedicated soft-surgery training was also recognized. A lack of agreement was found on whether the middle ear cavity should be flushed with a non-aminoglycoside antibiotic solution or whether a sheath or insertion tube should be used to avoid contaminating the array with blood or bone dust. In conclusion, this paper demonstrates how beliefs about CI soft surgery have changed since 2010 and shows areas of current consensus and disagreement.

A New Pathogenic Variant in POU3F4 Causing Deafness Due to an Incomplete Partition of the Cochlea Paved the Way for Innovative Surgery.

Incomplete partition type III (IP-III) is a relatively rare inner ear malformation that has been associated with a POU3F4 gene mutation. The IP-III anomaly is mainly characterized by incomplete separation of the modiolus of the cochlea from the internal auditory canal. We describe a 71-year-old woman with profound sensorineural hearing loss diagnosed with an IP-III of the cochlea that underwent cochlear implantation. Via targeted sequencing with a non-syndromic gene panel, we identified a heterozygous c.934G > C p. (Ala31Pro) pathogenic variant in the POU3F4 gene that has not been reported previously. IP-III of the cochlea is challenging for cochlear implant surgery for two main reasons: liquor cerebrospinalis gusher and electrode misplacement. Surgically, it may be better to opt for a shorter array because it is less likely for misplacement with the electrode in a false route. Secondly, the surgeon has to consider the insertion angles of cochlear access very strictly to avoid misplacement along the inner ear canal. Genetic results in well describes genotype-phenotype correlations are a strong clinical tool and as in this case guided surgical planning and robotic execution.

Randomized controlled trial of truncal exercises early after stroke to improve balance and mobility.

BACKGROUND: Sitting balance and the ability to perform selective truncal movements are important predictors of functional outcome after stroke. However, few clinical trials have evaluated the effect of truncal exercises. OBJECTIVE: The authors assessed the effect of additional truncal exercises on truncal function, standing balance, and mobility. METHODS: An assessor-blinded randomized controlled trial was carried out at a stroke rehabilitation hospital. A total of 33 participants (mean 35 days post onset) were randomly assigned to an experimental group (n = 18) or a control group (n = 15). In addition to conventional therapy, the experimental group received 16 hours of truncal exercises. The control group received 16 hours of sham treatment. Truncal function was evaluated by the Trunk Impairment Scale (TIS) and standing balance and mobility by the Tinetti Test. The Romberg with eyes open and eyes closed, Four Test Balance Scale (FTBS), Berg Balance Scale (BBS), Rivermead Motor Assessment Battery (RMAB), Functional Ambulation Categories, and Dynamic Gait Index (DGI) were performed to elucidate the findings of the primary outcome measures. RESULTS: A treatment effect was found for the experimental group on the TIS (P < .001), Tinetti Test (P < .001), FTBS (P = .014), BBS (P = .007), RMAB (P < .001), and DGI (P = .006). CONCLUSIONS: In addition to conventional therapy, truncal exercises have a beneficial effect on truncal function, standing balance, and mobility in people after stroke.

A prospective multicentre otology database.

UNLABELLED: WHY IS AN INTERNATIONAL OTOLOGY DATABASE IMPORTANT? There are many reports on the outcome of otological interventions in the literature. However, it is difficult to make direct comparisons between these reports because of the lack of uniformity in reporting outcomes and patient selection. The aim is to create an interactive otology database for surgeons in the UK and Europe. METHODOLOGY: The proposed project is the creation of a common database on a website. Two levels of data entry are available: (1) level I: minimum database (only main outcomes are included; for any otolaryngologist in Europe who wishes to join), and (2) level II: comprehensive database (detailed information on pathologies, risk factors and surgical procedure are also recorded; for invited otologists). As both databases share the same core data, clinicians using database I can still compare their outcome with those using database II. There has already been an international consensus on the content of the common otology database. A previous pilot study by 3 different hospitals also confirmed the user-friendliness of data input and retrieval using the web-based system. The system is now ready for international implementation. WHO MIGHT BENEFIT FROM THE DATABASE AND HOW? The size of the database allows statistical analysis to be made on various otological interventions with sufficient power. The web-based system can be a useful learning tool for surgeons because it gives instant feedback to the individual surgeon. It also provides the opportunity for hospitals to collaborate in clinical trials using this common data entry system.