Diabetes and Hearing Impairment: Knowledge, Attitudes, and Practices Among Providers and Patients.

PURPOSE: The purpose of this study was to assess the knowledge, attitudes, and practices among providers and patients regarding hearing impairment and screening referrals in people with diabetes. METHODS: A cross-sectional survey design among health care providers and patients at an academic medical center in Oklahoma was used to gather knowledge, attitudes, and practices data. RESULTS: Only 25.6% of providers selected hearing impairment as a complication of diabetes, whereas 96.7% selected retinopathy, kidney dysfunction, and foot infection. Reported barriers to referring patients for hearing impairment screenings were being unfamiliar with recommended screening frequency (57.3%) and existence of higher priorities (35.4%). When asked to select parts of the body affected by diabetes, 21.0% of patients surveyed selected ears, 88.0% selected feet, and 85.0% selected eyes and kidneys. Fewer patients reported being told hearing impairment is a complication of diabetes compared to retinopathy (8.1% vs 85.9%). Additionally, 24.2% of patients reported having a hearing impairment screening, and 96.0% reported having a dilated eye exam. CONCLUSIONS: Most providers and patients at an academic medical center are unaware of the relationship between diabetes and hearing impairment. Providers reported there are several barriers that need to be overcome to refer patients to audiologists.

Prolactinoma extension as a contributing factor in dopamine agonist-induced CSF rhinorrhea: a systematic review of the literature.

BACKGROUND: Dopamine agonist-induced cerebrospinal fluid (CSF) rhinorrhea is an uncommon treatment-related complication arising in 6.1% of prolactinoma patients treated with dopamine agonists. Locally invasive prolactinomas may create CSF fistulae through formation of dural and osseous skull base defects. Tumor shrinkage secondary to dopamine agonist therapy unmasks skull base defects, thus inducing CSF rhinorrhea. In these cases, repair of the leak may be achieved through collaborative surgical intervention by rhinologists and neurosurgeons. Multiple variables have been investigated as potential contributors to the risk of CSF rhinorrhea development in medically treated prolactinoma patients, with little consensus. OBJECTIVE: The primary aim of our study was the characterization of risk factors for CSF rhinorrhea development following dopamine agonist treatment. METHODS: A systematic review of the literature was conducted to identify cases of CSF rhinorrhea following dopamine agonist treatment of prolactinoma. The clinical history, radiographic findings and treatment outcomes are discussed. RESULTS: Fifty-four patients with dopamine agonist-induced CSF rhinorrhea were identified across 23 articles published from 1979 to 2019. Description of diagnostic imaging [computed tomography (CT)/magnetic resonance imaging (MRI)] was not provided for 18/54 subjects. For the 36 cases that described prolactinoma appearance on CT or MRI, invasion of the cavernous sinuses was reported in 13 (36.1%) and invasion of the sphenoid sinus was reported in 18 (50%). CONCLUSION: Based on our systematic review, we propose that CT findings of osseous erosion of the sella or the anterior skull base may predict dopamine agonist-induced CSF rhinorrhea. We recommend obtaining a thin-slice CT of the sinuses in cases with MRI evidence of sphenoid involvement.

Effect of CFTR modulator therapy on cystic fibrosis-related diabetes.

BACKGROUND: Half of adults with cystic fibrosis (CF) develop CF-related diabetes (CFRD). CFRD contributes to worsened pulmonary function and malnutrition. We undertook this study to determine the effect of cystic fibrosis transmembrane regulator (CFTR) modulators on CRFD. METHODS: We reviewed the medical records of adults with CF who followed in the CF clinic at Oklahoma University Medical Center. We collected data for age at diagnosis of CF and CFRD, CF mutations present, first date of ivacaftor therapy either alone or in combination, insulin use, pulmonary function, body mass index data, and home glucose monitoring results. Clinical resolution of CFRD was taken as discontinuation of routine insulin and resolution of high interstitial home glucose values. RESULTS: We identified 69 adult CF patients, of whom 31 had CFRD. Among these 14 CFRD patients taking ivacaftor alone or in combination, four patients completely stopped using insulin. Another patient went from three times a day pre-prandial insulin to using insulin once a week. Home blood glucose and hemoglobin A1c values supported resolution of CFRD. Three patients continued to have hypoglycemia despite stopping insulin. No CFRD patient not taking CFTR modulators markedly changed the insulin regimen. Pulmonary function was preserved in those patients with resolved CFRD (FEV1 +6.75% ±7.6), whereas it worsened in CFRD patients who either were not taking CFTR modulators (FEV1 -2.09% ±3.9) or who had no response of CFRD status (FEV1 -4.9% ±7.6). CONCLUSIONS: About one-third of patients on CFTR modulator therapy had resolution or near resolution of CFRD.

SPONTANEOUS CEREBROSPINAL FLUID LEAK AFTER INITIATION OF DOPAMINE AGONIST THERAPY IN MACROPROLACTINOMAS: TWO CASE REPORTS AND A LITERATURE REVIEW.

OBJECTIVE: To describe 2 spontaneous cerebrospinal fluid (CSF) leaks complicating treatment of macroprolactinoma (MPRL) with dopamine agonist (DA) therapy. METHODS: We present the 2 cases of spontaneous, DA-related CSF leaks. Prolactin levels were used to assess hyperprolactinemia. Beta-2 transferrin was tested in rhinorrhea fluid, and magnetic resonance imaging was used to assess the sella. RESULTS: Case 1 was a 45-year-old woman with a history of MPRL, recently started on bromocriptine at 15 mg/day, presented with clear rhinorrhea, headache, and nuchal rigidity. Magnetic resonance imaging showed a large sellar lesion extending into the cavernous sinuses, posterior sphenoid sinuses, and suprasellar cistern. Computed tomography revealed areas concerning for bony erosion, likely representing leak sites, and the rhinorrhea fluid was positive for beta-2 transferrin, confirming the CSF source. Empiric antibiotics for meningitis were given and she underwent urgent neuroendoscopic, transsphenoidal CSF leak repair and debulking of the pituitary mass. Case 2 was a 55-year-old man with a 10-year history of untreated MPRL who was started on bromocriptine at 5 mg/day 2 weeks prior to admission. He presented with clear rhinorrhea and a metallic taste in his mouth, worse with the Valsalva maneuver. Imaging confirmed clinical suspicion and he was taken for surgery. A high-flow CSF leak was encountered once the tumor was debulked. This was repaired with an abdominal fat pad graft. Both patients developed diabetes insipidus and required postoperative adjuvant DA therapy. CONCLUSION: Spontaneous CSF leaks can complicate medical therapy of large MPRL with underlying skull defects, typically within weeks of initiation of DA. This should prompt clinicians to educate patients about the symptoms of potential CSF rhinorrhea and encourage them to promptly report them.

Familial Pallister-Hall in adulthood.

Pallister Hall syndrome is autosomal dominant disorder usually diagnosed in infants and children. Current diagnostic criteria include presence of hypothalamic hamartoma, post axial polydactyly and positive family history, but the disease has variable manifestations. Herein we report Pallister Hall syndrome diagnosed in a family where both patients were adults. A 59 year old man developed seizures 4 years prior to our evaluation of him, at which time imaging showed a hypothalamic hamartoma. The seizures were controlled medically. He did well until he had visual changes after a traumatic head injury. Repeat MRI showed slight expansion of the mass with formal visual field testing demonstrating bitemporal hemianopsia. There was no evidence of pituitary dysfunction except for large urine volume. He underwent surgery to debulk the hamartoma and the visual field defects improved. There was no hypopituitarism post-operatively, and the polydyspia resolved. His 29 year old daughter also had seizures and hypothalamic hamartoma. Both patients had had polydactyly with prior surgical correction in childhood. The daughter underwent genetic testing, which revealed a previously undescribed heterozygous single base pair deletion in exon 13 of the GLI3 gene causing a frameshift mutation. Further investigation into family history revealed multiple members in previous generations with polydactyly and/or seizures. Pallister-Hall syndrome is caused by an inherited autosomal dominant or de novo mutation in GLI3 gene. This rare syndrome has not had prevalence defined, however. Generally, diagnoses are made in the pediatric population. Our report adds to the few cases detected in adulthood.